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1.  AB 36. Primary lung carcinoid, a rare cause of paraparesis: report of a case and review of the literature 
Journal of Thoracic Disease  2012;4(Suppl 1):AB36.
Carcinoids are neuroendocrine tumors involving the gastrointestinal tract, lungs and bronchi. They are usually benign with slow growth, but they can be malignant (metastasizing to liver, lungs, bones, skin, etc). Endocrine activity results in carcinoid syndrome: Intestinal carcinoid [5-hydroxytryptamine (serotonin) to portal circulation]: abdominal cramps, diarrhea, bronchospasm, face/chest cyanotic flushes, and right-sided stenotic cardiac lesions. Lung carcinoid (histamine and 5-hydroxytryptophan to systemic circulation): red facial flush/oedema, salivation, lachrymation, potentially tachycardia, fever, anxiety, tremor, hypotension, left-sided cardiac lesions). We present an extremely rare case of primary lung carcinoid presented with paraparesis.
Patient and methods
A 69 year old man developed progressively deteriorating bilateral proximal muscular weakness of the legs that within 6 months led to paraparesis (inability to stand up and walk) with preserved sensation. Electromyography showed polyneuropathy. History, clinical and laboratory data excluded diabetic mellitus, hypothyroidism, renal/liver failure, cachexia, alcohol abuse, chemotherapy, drug or other toxicity. Magnetic resonance imaging excluded spinal cord or spinal nerve root compression. Computed tomography revealed a small peripheral paracardiac right-middle lobe tumor (maximal diameter: 1.8cm). Thoracoscopic wedged resection was performed (endo-stappler). Histology revealed lung carcinoid of low mitotic index (1-2%).
The patient had an uneventful recovery, was discharged on the 5th postoperative day to a rehabilitation centre, where he remained for 3 months. Progressive motor function improvement was noted; paraparesis resolved completely within 6 months. At 1-year follow-up normal respiratory and neurological status was noted, in the absence of carcinoid recurrence or metastasis.
Myopathy is common in endocrine disease and cancer can have endocrine and metabolic activities and cause neurological complications. Encephalopathy, myelopathy, and more often motor and sensory neuropathy, myopathy, or neuromyopathy of central distribution may occur in paraneoplastic syndromes. Neuromyopathy has been reported in 14% of lung cancer patients. There are reports of recurrent facial palsy in metastatic thymic carcinoid, proximal muscle weakness in ACTH-releasing thymic carcinoid, an ACTH-releasing bronchial carcinoid, and paraparesis 40 years after pneumonectomy for carcinoid adenoma induced by inflammatory contents of the pneumonectomy cavity. To the best of our knowledge there is no report of paraparesis induced by primary pulmonary carcinoid. Complete resolution after excision suggests “paraneoplastic” syndrome.
PMCID: PMC3537365
2.  Imaging features of carcinoid tumors metastatic to the breast 
Cancer Imaging  2011;11(1):109-115.
The objective of this study was to describe the imaging findings of carcinoid tumors metastatic to the breast, with pathologic and clinical correlations. We searched our surgical database for cases of pathologically proven carcinoid tumors metastatic to the breast from October 1, 2000, to May 31, 2010. Of the approximate 10,000 breast biopsies identified, 7000 had malignant findings. Ten cases of metastatic carcinoid (0.1% of all malignancies), all with imaging studies available for review, were included in the study. All patients were women and had their primary carcinoid in the gastrointestinal tract (n=9) or lung (n = 1). One patient presented with a palpable breast mass and no history of carcinoid tumor; an ileal carcinoid was discovered after the pathologic diagnosis of metastatic carcinoid was established. In the breast, tumors presented as solitary lesions in half the cases. Metastases to the breast typically presented as circumscribed masses mammographically and as hypoechoic circumscribed masses ultrasonographically; some showed increased through-transmission and increased vascularity with color Doppler evaluation. Five patients had octreotide scans; of these, 4 had increased focal activity in the region of metastasis within the breast. Six patients underwent computed tomography. Without contrast, nodular masses were observed; with contrast, the masses showed rapid enhancement during arterial phase imaging. Magnetic resonance imaging (n = 4) also showed rapid enhancement and washout kinetics after contrast administration. Recognition of carcinoid metastases to the breast in patients with known or occult primary carcinoid tumors is important to avoid unnecessary treatment for primary breast cancer.
PMCID: PMC3205760  PMID: 21771708
Biopsy; pathology; treatment
3.  Atypical presentation of carcinoid tumor with unresolved right shoulder pain: a case report 
Carcinoid tumors are variants of neuroendocrine tumors that typically arise from the gastrointestinal tract and the bronchus, but they can involve any organ. Unresolved right shoulder pain manifesting as the first clinical presentation of carcinoid tumor with unknown primary origin is a rare clinical entity. To the best of our knowledge, herein we present the first case report describing metastasis to the right shoulder joint in a patient who presented with bone pain as the first clinical manifestation of metastatic carcinoid tumor of unknown primary origin. Metastasis to the right scapula as the first presentation of an underlying carcinoid tumor in the primary bronchus has been reported previously.
Case presentation
A 72-year-old Caucasian woman presented with pain in her right shoulder after a fall. She delayed seeking medical attention for 4 weeks for personal reasons. Her physical examination revealed no erythema or swelling of the right shoulder. However, tenderness was noted on the right subacromial bursa and the right acromioclavicular joint. Her drop arm test was positive. An X-ray of the right upper extremity showed no fracture. She did not respond to methylprednisolone injections or physical therapy. Because of the unresolved right shoulder pain with disturbance of her daily activities, magnetic resonance imaging of the right shoulder was ordered, which revealed permeative destruction of the right scapula. Because the permeative destruction of the bone could have been an osteolytic malignant feature, positron emission tomography–computed tomography was performed, which produced a scan showing osseous metastasis to the right scapula, multiple liver metastases and a 1.7cm right-lower-lobe pulmonary nodule. Her serotonin and chromogranin A levels were significantly elevated. The patient was treated with palliative cisplatin and etoposide chemotherapy followed by locoregional treatments for metastatic carcinoid tumor. She had mild improvement in her right shoulder pain, as well as better range of motion and improved quality of life, before she died less than 2 years after her diagnosis.
Our present case report emphasizes the protean manifestations of carcinoid tumors with the importance of early diagnosis of bone metastases from these tumors, because early diagnosis plays a major role in choosing the therapeutic regimen and prognosticating the course of the disease. The treatment goals for high-grade, poorly differentiated carcinoid tumors of unknown origin are decreasing the tumor load while controlling symptoms with chemotherapy and local modality treatments.
PMCID: PMC4031976  PMID: 24885024
Metastatic carcinoid tumor; Neuroendocrine tumor; Right shoulder pain
4.  Surgical treatment of a rare primary renal carcinoid tumor with liver metastasis 
Carcinoid tumors are characteristically low grade malignant neoplasms with neuroendocrine differentiation that arise in various body sites, most commonly the lung and gastrointestinal tract, but less frequently the kidneys, breasts, ovaries, testes, prostate and other locations. We report a case of a carcinoid of renal origin with synchronous single liver metastases on radiological studies.
Case presentation
A 45 year-old patient who presented with abdominal pain was found on CT scan to have lesions in the right ovary, right kidney, and left hepatic lobe. CA-125, CEA, and CA 19-9 were within normal limits, as were preoperative liver function tests and renal function. Biopsy of the liver mass demonstrated metastatic neuroendocrine tumor. At laparotomy, the patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, radical right nephrectomy with lymphadenectomy, and left hepatectomy. Pathology evaluation reported a right ovarian borderline serous tumor, well-differentiated neuroendocrine carcinoma of the kidney (carcinoid) with 2 positive retroperitoneal lymph nodes, and a single liver metastasis. Immunohistochemistry revealed that this lesion was positive for synaptophysin and CD56, but negative for chromogranin as well as CD10, CD7, and CD20, consistent with a well-differentiated neuroendocrine tumor. She is doing well one year after her initial surgery, with no evidence of tumor recurrence.
Early surgical intervention, together with careful surveillance and follow-up, can achieve successful long-term outcomes in patients with this rare malignancy.
PMCID: PMC2386474  PMID: 18430248
5.  Utility of the Serum ProGRP Level for Follow-up of Pulmonary Carcinoid Tumors 
Patient: Female, 67
Final Diagnosis: Pulmonary carcinoid tumor
Symptoms: Abnormal shadow on chest X-ray
Medication: —
Clinical Procedure: —
Specialty: Pulmonology
Rare disease
Although pulmonary carcinoid tumors are generally considered to represent a low-grade malignancy, atypical carcinoids are more aggressive than typical carcinoids, metastasizing more commonly to both regional lymph nodes and distant sites. The treatment of choice for localized disease is surgery. In cases of advanced or metastatic disease, medical treatments, including chemotherapy, have not been proven to be very successful. Therefore, providing careful follow-up is extremely important. In general, tumor markers, such as the level of CYFLA21-1, are often useful for monitoring lung cancer. However, there are currently no sensitive tumor markers for carcinoid tumors. We herein report a rare case of an atypical carcinoid of the lung with the elevation of the serum ProGRP level.
Case Eeport:
A 67-year-old female was referred to our hospital for an abnormal chest X-ray. CT revealed an 18×13 mm nodule in the right middle lobe with no significant mediastinal lymphadenopathy. The serum tumor marker, the ProGRP level, was significantly elevated (161 ng/ml). We performed a right middle lobectomy, because the pathological diagnosis of lung cancer was confirmed according to the results of a rapid frozen section biopsy of the lesion, although the pathological type could not be precisely determined by the frozen section alone. The final pathological diagnosis was atypical carcinoid. The level of ProGRP decreased (69 ng/ml) within 1 month after the surgery.
The ProGRP level may be useful for monitoring carcinoid tumors, although no serum tumor markers are highly specific or sensitive for detecting recurrences and/or distant metastasis of pulmonary carcinoid tumors. In conclusion, ProGRP should be further evaluated as biomarker in a larger series of patients to determine whether it demonstrates any significant correlation with cancer recurrence.
PMCID: PMC4138068  PMID: 25109370
Carcinoid Tumor; Lung Neoplasms; Tumor Markers, Biological
6.  Primary pulmonary carcinoid tumor with metastasis to endometrial polyp 
A carcinoid tumor occurring in the endometrium has been documented in the literature, but there is no report in regard to carcinoid tumor metastasis to endometrium.
We report a case of a malignant carcinoid metastasis to an endometrial polyp. Patient underwent hysteroscopy, and polypectomy. The pathology demonstrated an endometrial polyp containing a 4 mm x 5 mm nodule of metastatic carcinoid tumor, consistent with metastasis from patient's known pulmonary carcinoid. The tumor was morphologically similar to the tumors of the right lung, with similar immune-profile.
This patient presented with a suspicious pelvic ultrasound. Due to her age, the first priority was to exclude uterine cancer. The endometrial polyp, which was found, had a small focus of metastatic carcinoid tumor. To the best of our knowledge, this finding has not been previously recorded in the literature. Our patient also had a history of metastatic carcinoid tumor to breast. This finding is also very uncommon.
This is the first case in the literature described a malignant carcinoid metastasis to an endometrial polyp.
PMCID: PMC3537946  PMID: 23127865
Metastatic carcinoid tumor; Endometrial polyp
7.  Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera 
Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501000/µL, and leukocytosis of 20410/µL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.
PMCID: PMC4316038  PMID: 15968742
Carcinoid tumor; Accessory papilla; Polycythemia vera
8.  Primary lung carcinoid, a rare cause of paraparesis: report of a case and review of the literature 
Journal of Thoracic Disease  2012;4(Suppl 1):49-55.
Carcinoids are neuroendocrine tumors mainly involving the gastrointestinal tract, lungs and bronchi. They were considered benign with slow growth, but they can be malignant in a substantial percentage of patients (metastasizing to liver, bones, skin, etc). Endocrine activity results in carcinoid syndrome. Proximal myopathy has been reported in 7% of patients with carcinoid syndrome. Bronchopulmonary and thymic carcinoids producing adrenocorticotropic hormone can cause Cushing’s syndrome, a main feature of which is myopathy. There are a few reports of carcinoids associated with paraneoplastic neurological syndromes, including neuropathy. We hereby present an extremely rare case of a primary lung carcinoid presented with paraparesis due to polyneuropathy, and review the relevant literature. To the best of our knowledge there is no similar previous report. Complete resolution of paraparesis after excision of the lung carcinoid suggests paraneoplastic neurological syndrome.
PMCID: PMC3537397  PMID: 23304441
Carcinoid; carcinoid tumor/complications; paraneoplastic neurological syndrome; paraneoplastic syndromes/etiology; polyneuropathy; paraparesis
9.  Hypergastrinemia and recurrent type 1 gastric carcinoid in a young Indian male: Necessity for antrectomy? 
Carcinoid tumors are the most common neuroendocrine tumors. Gastric carcinoids represent 2% of all carcinoids and 1% of all gastric masses. Due to the widespread use of Esophagogastroduodenoscopy for evaluating a variety of upper gastrointestinal symptoms, the detection of early gastric carcinoids has increased. We highlight an alternative management of a young patient with recurrent type 1 gastric carcinoids with greater than 5 lesions, as well as lesions intermittently greater than 1 cm. Gastric carcinoids have a variable presentation and clinical course that is highly dependent on type. Type 1 gastric carcinoids are usually indolent and have a metastasis rate of less than 2%, even with tumors larger than 2 cm. There are a number of experts as well as organizations that recommend endoscopic resection for all type 1 gastric carcinoid lesions less than 1 cm, with a follow-up every 6-12 mo. They also recommend antrectomy for type 1 gastric carcinoids with greater than 5 lesions, lesions 1 cm or greater, or refractory anemia. However, the American Society of Gastrointestinal Endoscopy guidelines state that type 1 gastric carcinoid surveillance is controversial based on the evidence and could not make an evidence-based position statement on the best treatment modality. Our report illustrates a rare cause of iron deficiency anemia in a young male (without any medical history) due to multiple recurrent gastric carcinoid type 1 lesions in the setting of atrophic gastritis causing hypergastrinemia, and in the absence of a vitamin B12 deficiency. Gastric carcinoid type 1 can present in young males without an autoimmune history, despite the known predilection for women aged 50 to 70 years. Type 1 gastric carcinoids can be managed by endoscopic resection in patients with greater than 5 lesions, even with lesions larger than 1 cm. This course of treatment enabled the avoidance of early antrectomy in our patient, who expressed a preference against more invasive measures at his young age.
PMCID: PMC3199567  PMID: 22046097
Gastric carcinoid; Antrectomy; Endoscopic resection; Hypergastrinemia; Iron deficiency anemia
10.  Synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney: a unique case report and review of the literature 
Diagnostic Pathology  2009;4:17.
Malignant transformation of mature cystic teratoma is a rare complication. While any of the constituent tissues of a teratoma has the potential to undergo malignant transformation, squamous cell carcinoma is the most commonly associated malignancy. Renal carcinoid tumors are rare and frequently associated with horseshoe kidney and renal teratoma. Renal teratoma rarely presents together with carcinoid tumor or adenocarcinoma. To the best of our knowledge, there has never been a report of renal teratoma coexisting with both carcinoid tumor and adenocarcinoma.
Here, we present a unique and first case of synchronous primary carcinoid tumor and moderately differentiated adenocarcinoma arising within mature cystic teratoma of horseshoe kidney in a 50-year-old female. Lumbar spine X-ray, done for her complaint of progressive chronic low back pain, accidentally found a large calcification overlying the lower pole of the right kidney. Further radiologic studies revealed horseshoe kidney and a large multiseptated cystic lesion immediately anterior to the right renal pelvis with central calcification and peripheral enhancement. She underwent right partial nephrectomy.
Macroscopically, the encapsulated complex solid and multiloculated cystic tumor with large calcification, focal thickened walls and filled with yellow-tan gelatinous material. Microscopically, the tumor showed coexistent mature cystic teratoma, moderately differentiated adenocarcinoma and carcinoid tumor. Immunohistochemically, alpha-methylacyl-coenzyme A-racemase, calretinin, CD10 and thyroid transcription factor-1 were negative in all the three components of the tumor. The teratomatous cysts lined by ciliated epithelium showed strong staining for cytokeratin 7 and pancytokeratin, and those lined by colonic-like epithelium showed strong staining for CDX2, cytokeratin 20 and pancytokeratin, but both were negative for calretinin. Additionally, the teratomatous cyst wall showed strong staining for smooth muscle actin, and weak staining for carbonic anhydrase IX, CD99, chromogranin and synaptophysin. The adenocarcinoma component was strongly positive for cytokeratin 7 and pancytokeratin, weakly positive for synaptophysin and CD56, and negative for carbonic anhydrase IX, CD99, CDX2, chromogranin, cytokeratin 20 and smooth muscle actin. The carcinoid tumor component was strongly positive for CD56, chromogranin and synaptophysin, weakly positive for pancytokeratin, and negative for carbonic anhydrase IX, CD99, CDX2, cytokeratin 7, cytokeratin 20 and smooth muscle actin. She received no adjuvant therapy and is alive without evidence of disease six months after diagnosis and surgery.
This unique and first case herein presented with synchronous primary carcinoid tumor and primary adenocarcinoma arising within mature cystic teratoma of horseshoe kidney emphasizes the need for thorough sectioning and entire submission for histologic evaluation of mature cystic teratomas, in order to avoid missing multiple additional histogenetically distinct neoplasms.
PMCID: PMC2704177  PMID: 19523243
11.  Primary Carcinoid Tumour of the Kidney: A Review of the Literature 
Advances in Urology  2013;2013:579396.
Context. Primary renal carcinoid tumours are rare. Their pathogenesis is unknown and the clinical presentation is similar to other renal tumours thus posing diagnostic dilemmas for clinicians. Objectives. To review the literature for case reports of primary renal carcinoids. Methods. Literature was extensively searched for case reports for primary renal carcinoids. Reports of metastatic carcinoids to the kidneys were excluded. Results. Approximately less than 90 cases of primary carcinoid tumours of the kidney have been reported in the literature. A total of 29 cases of primary renal carcinoids were reviewed. The mean age of presentation was 48 years (range 29–75) with both right kidney (48.3%) and left kidney (44.8%) being equally affected. 28.6% of the cases reviewed were diagnosed as an incidental finding. The mean followup time was 20 months with 73.1% of patients without evidence of disease after surgical treatment (radical or partial nephrectomy). Primary carcinoid tumours of the kidney are often well differentiated tumours. They are often misdiagnosed because of their rarity and similar presentation with other renal tumours. Conclusions. Primary carcinoid tumours of the kidney are rare tumours with an indolent course with frequent metastasis. Metastatic work up and followup is required in their management.
PMCID: PMC3755439  PMID: 23997766
12.  Prognosis and risk factors of metastasis in colorectal carcinoids: results of a nationwide registry over 15 years 
Gut  2007;56(6):863-868.
Colorectal carcinoids are often described as low‐grade malignant. However, no study has compared the survival between patients with colorectal carcinoids and those with carcinomas, in a large series. In addition, no global consensus has been established on the crucial determinants of metastasis in colorectal carcinoids.
To determine the predictive factors for metastasis in colorectal carcinoids and clarify their prognosis compared with adenocarcinomas.
Data of all patients diagnosed as having colorectal carcinoids were extracted from a large nationwide database of colorectal tumours, the Multi‐Institutional Registry of Large‐Bowel Cancer in Japan, for the period from 1984 to 1998. Risk factors for lymph node (LN) metastases and distant metastases were analysed among those who were undergoing surgery, by univariate and multivariate analysis. Cancer‐specific survival was also compared between patients with colorectal carcinoids and those with adenocarcinomas registered in the same period.
Among the 90 057 cases of colorectal tumours that were diagnosed, a total of 345 cases of carcinoids were identified, including 247 colorectal carcinoids of those undergoing surgery. Risk factors for LN metastasis were tumour size ⩾11 mm and lymphatic invasion, whereas those for distant metastasis were tumour size ⩾21 mm and venous invasion. Colorectal carcinoids without these risk factors exhibited no LN metastasis or distant metastasis. Cancer‐specific survival of patients with colorectal carcinoids without metastasis was better than that of those with adenocarcinomas. However, the survival was similar between carcinoids and adenocarcinomas if the tumours had LN metastasis or distant metastasis.
The presence of metastasis in colorectal carcinoids could lead to survival that is as poor as in adenocarcinomas. Tumours ⩽10 mm and without lymphatic invasion could be curatively treated by local resection, but others would need radical LN dissection.
PMCID: PMC1954860  PMID: 17213340
13.  Primary carcinoid tumor arising within mature teratoma of the kidney: report of a rare entity and review of the literature 
Diagnostic Pathology  2007;2:15.
Primary carcinoid tumor arising within mature teratoma of the kidney is extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary carcinoid tumor arising within mature teratoma of the kidney.
Six previously reported case reports were identified using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. We also searched the electronic medical archival records of our institution and identified one additional unreported case. Data were extracted on the demographics, predisposing factors, clinical presentation, radiographic features, gross pathology, microscopic pathology, immunophenotype, therapy, and outcome of each of these seven cases.
Primary carcinoid tumor arising within mature teratoma of the kidney was found at a mean age of 41.4 years. Of the 7 cases, 3 were female and 4 were male. Two of the 7 cases (28.6%) were associated with horseshoe kidney. It typically presented with abdominal pain without carcinoid syndrome. It typical radiologic appearance was well circumscribed partly calcified Bosniak II-III lesion. Histologically, the carcinoid tumor showed monotonous small round cells arranged in classic anastomosing cords/ribbons intermixed with solid nests. Surgery was curative, no additional treatment was required, no local recurrences occurred, and no metastases occurred in all 7 cases. The 3 cases with available outcome data were alive at the time of publication of their respective cases (mean, 5 months).
Primary carcinoid tumor arising within mature teratoma of the kidney is a rare tumor that typically presents with abdominal pain without carcinoid syndrome. It is not associated with local recurrence and metastasis, is surgically curable, and has excellent prognosis.
PMCID: PMC1884130  PMID: 17509135
14.  Gastric Carcinoid Tumors 
The Ochsner Journal  2008;8(4):191-196.
Initially, carcinoid tumors were a curiosity for physicians and were so named because of their relatively benign behavior as compared to the more common adenocarcinomas. As medicine has evolved, our understanding and management have greatly improved. Our classification system has also become more specific. Gastric carcinoid tumors are unique in that three types have been described based upon each one's pathophysiology. In general, none of these give rise to the typical carcinoid syndrome as seen with metastatic ileal carcinoids.
Type 1 gastric carcinoids represent 70% to 80% and are characterized by multiple small lesions and their association with hypergastrinemia secondary to chronic atrophic gastritis and pernicious anemia, and are less likely to metastasize. Type 2 is a rare entity, representing 5%, and is characterized by multiple small lesions, hypergastrinemia secondary to Zollinger-Ellison syndrome, and multiple endocrine neoplasia (MEN) type 1. The risk for metastasis is slightly higher than for type 1; however, overall prognosis is dependent upon the gastrinoma prognosis. Accounting for 20%, type 3 is known as sporadic gastric carcinoids in that there is no association with hypergastrinemia, chronic atrophic gastritis, or Zollinger-Ellison syndrome. These present as large solitary lesions and are often metastatic upon diagnosis. A unique feature of type 3 is its association with an atypical carcinoid syndrome that is thought to be mediated by histamine.
The treatment—including medical, endoscopic, and surgical—of gastric carcinoids is dictated by the type, size, and presence of metastasis.
PMCID: PMC3096380  PMID: 21603501
chronic atrophic gastritis; Gastric carcinoid; GEP-NETs; hypergastrinemia; multiple endocrine neoplasia (MEN) type 1; pernicious anemia; somatostatin; somatostatin receptor scintigraphy; Zollinger-Ellison syndrome
15.  Clinical symptoms, hormone profiles, treatment, and prognosis in patients with gastric carcinoids 
Gut  1998;43(2):223-228.
Background—Type 1 gastric carcinoids are associated with hypergastrinaemia and chronic atrophic gastritis, type 2 occur in patients with multiple endocrine neoplasia type 1 combined with Zollinger-Ellison syndrome, and type 3 lack any relation to hypergastrinaemia. Type 1 tumours are usually benign whereas type 3 are highly malignant. 
Aims—To identify possible tumour markers in patients with gastric carcinoids. 
Patients/method—Nine patients with type 1, one with type 2, and five with type 3 were evaluated with regard to symptoms, hormone profile, and prognosis. 
Results—Plasma chromogranin A was increased in all patients but was higher (p<0.01) in those with type 3 than those with type 1 carcinoids. All patients with type 3 carcinoids died from metastatic disease, but none of the type 1 patients died as a result of their tumours. One type 1 patient with a solitary liver metastasis received interferon α and octreotide treatment. Nine months later, the metastasis was no longer detectable. She is still alive eight years after diagnosis, without recurrent disease. This represents the only reported case of foregut carcinoid with an unresectable liver metastasis that seems to be have been cured by biotherapy. 
Conclusions—Plasma chromogranin A appears to be a valuable tumour marker for all types of gastric carcinoid. Combination therapy with interferon α and octreotide may be beneficial in patients with metastasising type 1 gastric carcinoids. 

Keywords: gastric carcinoids; chromogranin; histamine; interferon; octreotide
PMCID: PMC1727202  PMID: 10189848
16.  Skeletal metastasis of carcinoid tumors: Two case reports and review of the literature 
Oncology Letters  2012;3(5):1105-1108.
Carcinoid tumors are neuroendocrine neoplasms derived from enterochromaffin cells. Skeletal metastases from carcinoid tumors are considered to be extremely rare. In this study, we present two cases of carcinoid tumors that metastasized to the bone. Furthermore, we review 50 published case reports and reveal the features of skeletal metastasis of carcinoid tumors. The first case involved a 59-year-old man with a history of multiple metastases of a lung carcinoid tumor. The patient complained of back pain and numbness in the lower limbs. Magnetic resonance imaging revealed metastases in the thoracic spine. A spinal decompression was performed and the symptoms were resolved. The second case involved a 74-year-old man had been diagnosed with a lung carcinoid tumor 15 years previously and complained of left thigh pain. A radiograph showed osteolytic lesions in the shaft of the left femur. We repaired the femur using an intramedullary nail following curettage of the tumor. A radiograph of the femur revealed a callus on the pathological fracture. The patient was able to walk using a crutch 3 months after surgery. We reviewed 50 cases that described skeletal metastasis from carcinoid tumors. The average age of the patients was 54.9 years and 33 patients (66%) were male. The most common site of skeletal metastasis was the spine. We also investigated the survival rate of patients who developed skeletal metastasis from carcinoid tumors. The findings showed that survival of patients who developed osteolytic skeletal metastasis was worse than that of patients who developed osteosclerotic skeletal metastasis.
PMCID: PMC3389625  PMID: 22783400
carcinoid tumor; skeletal metastasis; prognosis
17.  Primary renal carcinoid tumour with inferior vena caval tumour thrombus 
Carcinoid tumours, most frequently reported in the gastrointestinal and respiratory tracts, are exceedingly rare primary renal cancers. Few cases have been published to date. To our knowledge, we report the first case of a primary carcinoid tumour of the kidneys involving the inferior vena cava. We treated a 58-year-old woman with an open radical nephrectomy and cavotomy with thrombectomy. We describe the presentation, investigations and pathology results. We discuss the current experience with carcinoid tumours as a literature review relating to the diagnosis of the disease and the prognosis of patients with this neoplasm. Localized carcinoid tumours of the kidneys, including those involving the vena cava, can be successfully treated with surgical excision.
PMCID: PMC2692164  PMID: 19543456
18.  Primary hepatic carcinoid: A case report and literature review 
Carcinoids are tumors derived from neuroendocrine cells and often produce functional peptide hormones. Approximately 54.5% arise in the gastrointestinal tract and frequently metastasize to the liver. Primary hepatic carcinoid tumors (PHCT) are extremely rare; only 95 cases have been reported. A 65-year-old man came to our attention due to occasional ultrasound findings in absence of clinical manifestations. His previous medical history, since 2003, included an echotomography of the dishomogeneous parenchymal area but no focal lesions. A computed tomography scan performed in 2005 showed an enhanced pseudonodular-like lesion of about 2 cm. Cholangio-magnetic resonance imaging identified the lesion as a possible cholangiocarcinoma. No positive findings were obtained with positron emission tomography. Histology suggested a secondary localization in the liver caused by a low-grade malignant neuroendocrine tumor. Immunohistochemistry was positive for anti chromogranin antibodies, Ki67 antibodies and synaptophysin. Octreoscan scintigraphy indicated intense activity in the lesion. Endoscopic investigations were performed to exclude the presence of extrahepatic neoplasms. Diagnosis of PHCT was established. The patient underwent left hepatectomy, followed by hormone therapy with sandostatine LAR. Two months after surgery he had a lymph nodal relapse along the celiac trunk and caudate lobe, which was histologically confirmed. The postoperative clinical course was uneventful, with a negative follow-up for hematochemical, clinical and radiological investigations at 18 mo post-surgery. Diagnosis of PHCT is based principally on the histopathological confirmation of a carcinoid tumor and the exclusion of a non-hepatic primary tumor. Surgical resection is the recommended primary treatment for PHCT. Recurrence rate and survival rate in patients treated with resection were 18% and 74%, respectively.
PMCID: PMC2684614  PMID: 19452590
Carcinoid; Primary hepatic carcinoid; Neuroendocrine neoplasm; Therapy; Surgical treatment; Prognosis
19.  Breast metastasis of ilial carcinoid tumor: Case report and literature review 
Metastatic breast carcinoids are rare neoplasms. They can be mistaken for primary breast carcinoma both clinically and radiologically, even with known history of carcinoid tumor elsewhere in the body.
Case presentation
We report a case of unilateral breast metastasis from carcinoid tumor of the small intestine in a 52-year-old woman who was successfully treated by lumpectomy and radiation therapy. An extensive review of the literature reveals only a few cases of metastatic carcinoid to the breast from small intestinal primaries.
Clinical suspicion for metastasis should be high in a patient with breast mass and history of known carcinoid elsewhere in the body. Lumpectomy alone may be effective in these patients. Mastectomy and especially axillary dissection could be avoided. Their histological appearance may mimic ductal adenocarcinoma of the breast. However, the distinction is important due to differences in management and prognosis.
PMCID: PMC1481627  PMID: 16566835
20.  Goblet cell carcinoid of the appendix 
Goblet cell carcinoid (GCC) of the appendix is a rare neoplasm that share histological features of both adenocarcinoma and carcinoid tumor. While its malignant potential remains unclear, GCC's are more aggressive than conventional carcinoid. The clinical presentations of this neoplasm are also varied. This review summarizes the published literature on GCC of the appendix. The focus is on its diagnosis, histopathological aspects, clinical manifestations, and management.
Published studies in the English language between 1966 to 2004 were identified through Medline keyword search utilizing terms "goblet cell carcinoid," "adenocarcinoid", "mucinous carcinoid" and "crypt cell carcinoma" of the appendix.
Based on the review of 57 published papers encompassing nearly 600 diagnosed patients, the mean age of presentation for GCC of the appendix was 58.89 years with equal representation in both males and females. Accurate diagnosis of this neoplasm requires astute observations within an acutely inflamed appendix as this neoplasm has a prominent pattern of submucosal growth and usually lacks the formation of a well-defined tumor mass. The mesoappendix was involved in 21.64% followed by perineural involvement in 2.06%. The most common clinical presentations in order of frequency were acute appendicitis in 22.5%; asymptomatic in 5.4%; non-localized abdominal pain in 5.15% and an appendicular mass in 3.09%. The most common surgical treatment of choice was appendectomy with right hemicolectomy in 34.70% followed by simple appendectomy in 24.57%. Concomitant distant metastasis at diagnosis was present in 11.16% of patients with the ovaries being the most common site in 3.60% followed by disseminated abdominal carcinomatosis in 1.03%. Local lymph node involvement was seen in 8.76% of patients at the time of diagnosis. The reported 5-year survival ranges from 60 % to 84%. GCC's of the appendix remains a neoplasm of unpredictable biological behavior and thus warrants lifelong surveillance for recurrence of the disease upon diagnosis and successful surgical extirpation.
GCC of the appendix is a rare neoplasm. Due to its wide range of presentation, this tumor should be considered as a possible diagnosis in many varied situations leading to abdominal surgery. Histopathological features such as increased number of Paneth cells, increased amount of mucin secretion and presence of pancreatic polypeptide may predict a more aggressive behavior. The advocated plan of management recommended for patients with tumors that involve the adjacent caecum or with high-grade tumors with histological features such as an increased mitotic rate involve initial appendectomy with completion right hemicolectomy due to the high possibility of local recurrence with intraperitoneal seeding prior to lymph node involvement and a 20% risk of metastatic behavior. In female patients with GCC of the appendix regardless of age, bilateral salpingo-oophorectomy is advocated. In cases with obvious spread of the disease chemotherapy, mostly with 5-FU and leucovorin is advised. Cytoreductive surgery with adjuvant intraperitoneal chemotherapy can offer improved survival in cases with advanced peritoneal dissemination.
PMCID: PMC1182398  PMID: 15967038
21.  Clinicopathologic characteristics of colonic carcinoid tumors 
The Journal of surgical research  2013;184(1):183-188.
Extraappendiceal colonic carcinoids are uncommon neuroendocrine tumors with a poor prognosis compared to carcinoids of other gastrointestinal origins. Few studies have examined the clinicopathologic profile and behavior of this rare tumor.
Materials and Methods
A retrospective analysis was performed on patients with colonic carcinoid tumors evaluated at a single tertiary care center between 1996 and 2012. Collected data included patient and tumor characteristics, presentation, treatment, recurrence and survival. Results were integrated into a comprehensive review of the colonic carcinoid literature.
In total, 114 patients with colorectal carcinoid tumors were identified, and 15 patients with extraappendiceal tumors were analyzed. The mean age was 58.6±3.0 years, and subjects were predominantly male (73.3%). The most common presenting complaint was abdominal pain (33.3%), although 26.7% of patients were asymptomatic. Cecal tumors were the most prevalent (73.3%), and the majority of patients underwent right hemicolectomy. Three patients with lesions <1 cm were treated endoscopically. The mean tumor diameter was 2.9±0.5 cm, with lymph node or distant metastasis present in 53.3% and 26.7%, respectively. All but 2 patients underwent a presumed curative resection. During a mean follow-up of 4.2±1.0 years, there was only one death (non-carcinoid specific). Eleven patients were alive without evidence of disease at last follow-up, and 3 patients were alive with disease, one of which initially had a presumed curative resection but recurred.
This case series further elucidates the clinicopathologic characteristics of colonic carcinoid tumors, which aids physicians in guiding the diagnosis and management of these rare tumors.
PMCID: PMC3759590  PMID: 23830363
22.  Multiple rectal carcinoids with diffuse ganglioneuromatosis 
Rectal carcinoids comprise only about 1% of all anorectal neoplasms. In addition, ganglioneuroma of the gastrointestinal tract is a rare tumor composed ganglion cells, nerve fibers, and supporting cells. Multiple carcinoid tumors with diffuse ganglioneuromatosis limited to the rectum are quite unusual.
Case presentation
A 69-year-old man was referred to us because of about 100 small submucosal rectal tumors. He underwent abdominoperineal resection. Pathology revealed carcinoid tumors for about 30 submucosal nodules and diffuse ganglioneuromotosis. To date (6 months later) he remains well with no recurrence.
Although the optimal treatment for the multiple rectal carcinoids remains to be clearly established, it is believed that not all patients with multiple rectal carcinoids (measuring less than 1 cm in diameter) need to have a radical operation. However, the treatment plan for each case should be individualized and a careful follow-up is mandatory.
PMCID: PMC1805501  PMID: 17306015
23.  Clinical Characteristics of Colorectal Carcinoid Tumors 
The carcinoid tumor was recently categorized as a malignant disease due to its possibility of metastasis. This study was aimed to investigate the clinical characteristics and the metastatic rate of colorectal carcinoid tumors.
Charts were reviewed for 502 patients diagnosed with and treated for colorectal carcinoid tumors between January 2006 and December 2009. The location, size, depth and metastatic status of the tumors were collected.
Including 24 synchronous tumors from 12 patients, 514 carcinoid tumors were removed. Most of them were found in the rectum (97.3%). The male-to-female ratio was 1.38 to 1, and mean age was 50.2 ± 11.4 years. The mean tumor size was 5.8 ± 3.6 mm. Less than 10-mm-sized tumors had a 1.95% lymph node metastatic rate; tumors with sizes from 10 mm to 20 mm and larger than 20 mm had 23.5% and 50% lymph node metastatic rates, respectively. Two cases had distant metastasis; one with a 22-mm-sized tumor metastasized to the liver, and the other with a 20-mm-sized tumor metastasized to the peritoneum. Among 414 patients who completed metastatic studies, 93.8% were classified as stage I, 0.9% as stage II, 4.8% as stage III and 0.5% as stage IV.
Colorectal carcinoid tumors smaller than 10 mm have a low rate of lymph node metastasis, but those sized 10 mm or larger incur significant risk. Further investigation regarding additional risk factors should be done to develop proper treatment guidelines for these tumors.
PMCID: PMC3053496  PMID: 21431092
Carcinoid tumor; Colorectal carcinoid tumor; Lymph node metastasis
24.  Delayed Recurrence of Atypical Pulmonary Carcinoid Cluster: A Rare Occurrence 
Case Reports in Pulmonology  2014;2014:620814.
Carcinoid is one of the most common tumors of the gastrointestinal tract followed by the tracheobronchial tree. Bronchial carcinoid compromises 20% of total carcinoid and accounts for 1–5% of pulmonary malignancies. Carcinoid can be typical or atypical, with atypical carcinoid compromises 10% of the carcinoid tumors. Carcinoid usually presents as peripheral lung lesion or solitary endobronchial abnormality. Rarely it can present as multiple endobronchial lesion. We hereby present a rare case of an elderly gentleman who had undergone resection of right middle and lower lobe of lung for atypical carcinoid. Seven years later he presented with cough. CT scan of chest revealed right hilar mass. Flexible bronchoscopy revealed numerous endobronchial polypoid lesions in the tracheobronchial tree. Recurrent atypical carcinoid was then confirmed on biopsy.
PMCID: PMC4251887  PMID: 25506019
25.  Typical laryngeal carcinoid tumor with recurrence and lymph node metastasis: a case report and review of the literature 
Typical carcinoid tumor of the larynx is an extremely rare lesion which arises from neuroendocrine cells scanning in the laryngeal mucosa or submucosal glands. Conventionally, it is a well-differentiated neuroendocrine carcinoma, conservative surgery represents the treatment of choice, and the patient usually has a good prognosis with rarely recurrence and metastasis. In this report, we present a case of typical laryngeal carcinoid tumor with recurrence and lymph node metastasis. The patient was a 58-year-old man, complaints of intermittent burning pain in his both ears for 2 years, and for the recent one month the pain turn to continuous accompanied with a mild discomfort in the throat, he had neither hoarseness, dysphagia, nor any otorrhea and hearing loss. The patient was scheduled to undergo a tracheotomy and then a biopsy under supporting laryngoscopy. During the operation, the frozen section diagnosis from the first and the second time both indicated that the biopsy specimens originated from musculo-epithelia, it couldn’t be differed from malignant to benign. So the mass was simply removed under supporting laryngoscopy. The histopathology from paraffin sections revealed typical carcinoid of the larynx and the second procedure consisted of supraglottic laryngectomy with clear margins, the otalgia resolved and the patient had no difficulty with phonation or swallowing. After 1 year follow-up, the patient was found a mass on his right neck with symptom free, B-ultrasonography indicated several enlargement lymph nodes with some merged on both sides of the neck, the patient was scheduled to undergo a “total-laryngectomy with radical neck dissection on the left side and an elective neck dissection on the right side”. The specimens were positive for cytokeratin, chromogranin A and synaptophysin, a final diagnosis of typical carcinoid was made for the recurrence lesion and the metastasis of the lymph nodes. Though the post-operative recovery was uneventful, the prognosis was not good, the patient died six months later. Here, we review the pertinent references on this subject, and discuss the main managements for typical carcinoid tumor of the larynx.
PMCID: PMC4314005
Typical carcinoid; larynx; recurrence; metastasis

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