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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.
Background
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%).
Results
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.
Conclusions
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.
doi:10.3978/j.issn.2072-1439.2012.s036
PMCID: PMC3537365
2.  Synchronous Adenocarcinoma of the Colon and Rectal Carcinoid 
Case Reports in Gastroenterology  2016;10(3):600-604.
Primary colonic adenocarcinoma and synchronous rectal carcinoids are rare tumors. Whenever a synchronous tumor with a nonmetastatic carcinoid component is encountered, its prognosis is determined by the associate malignancy. The discovery of an asymptomatic gastrointestinal carcinoid during the operative treatment of another malignancy will usually only require resection without additional treatment and will have little effect on the prognosis of the individual. This article reports a synchronous rectal carcinoid in a patient with hepatic flexure adenocarcinoma. We present a case of a 46-year-old Hispanic woman with a history of hypothyroidism, uterine fibroids and hypercholesterolemia presenting with a 2-week history of intermittent abdominal pain, mainly in the right upper quadrant. She had no family history of cancers. Physical examination was significant for pallor. Laboratory findings showed microcytic anemia with a hemoglobin of 6.6 g/dl. CT abdomen showed circumferential wall thickening in the ascending colon near the hepatic flexure and pulmonary nodules. Colonoscopy showed hepatic flexure mass and rectal nodule which were biopsied. Pathology showed a moderately differentiated invasive adenocarcinoma of the colon (hepatic flexure mass) and a low-grade neuroendocrine neoplasm (carcinoid of rectum). The patient underwent laparoscopic right hemicolectomy and chemotherapy. In patients diagnosed with adenocarcinoma of the colon and rectum, carcinoids could be missed due to their submucosal location, multicentricity and indolent growth pattern. Studies suggest a closer surveillance of the GI tract for noncarcinoid synchronous malignancy when a carcinoid tumor is detected and vice versa.
doi:10.1159/000450677
PMCID: PMC5121549  PMID: 27920648
Synchronous tumors; Adenocarcinoma of colon; Rectal carcinoid
3.  Imaging features of carcinoid tumors metastatic to the breast 
Cancer Imaging  2011;11(1):109-115.
Abstract
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.
doi:10.1102/1470-7330.2011.0018
PMCID: PMC3205760  PMID: 21771708
Biopsy; pathology; treatment
4.  Atypical presentation of carcinoid tumor with unresolved right shoulder pain: a case report 
Introduction
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.
Conclusion
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.
doi:10.1186/1752-1947-8-142
PMCID: PMC4031976  PMID: 24885024
Metastatic carcinoid tumor; Neuroendocrine tumor; Right shoulder pain
5.  Surgical treatment of a rare primary renal carcinoid tumor with liver metastasis 
Background
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.
Conclusion
Early surgical intervention, together with careful surveillance and follow-up, can achieve successful long-term outcomes in patients with this rare malignancy.
doi:10.1186/1477-7819-6-41
PMCID: PMC2386474  PMID: 18430248
6.  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
Objective:
Rare disease
Background:
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.
Conclusions:
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.
doi:10.12659/AJCR.890692
PMCID: PMC4138068  PMID: 25109370
Carcinoid Tumor; Lung Neoplasms; Tumor Markers, Biological
7.  Carcinoid Tumors of Digestive Organs: a Clinicopathologic Study of 13 Cases 
Gastroenterology Research  2009;2(1):35-37.
The author investigated clinicopathologic features of 13 cases of carcinoid tumor in the digestive organs. The 13 cases of carcinoid were identified from 18,267 pathological specimens of digestive organs in the last 10 years in our pathology laboratory. The tumor locations were rectum in 9 cases, duodenum in 2 cases, liver in 1 case, and stomach in 1 case. The age of the patients ranged from 52 to 82 years with a mean of 63 years. Male to female ratio was 7 : 6. The presenting symptoms were abnormal pain in 3 cases and asymptomatic in 10 cases. None of the cases showed carcinoid syndrome. The diameter ranged from 5 mm to 25 mm in gastrointestinal carcinoids, and 60 mm in the hepatic carcinoid. The treatment was endoscopic mucosal resection in 10 cases and surgical resection in 3 cases. The outcome is good except for hepatic atypical carcinoid which showed metastases and died of systemic metastasis. Histologically, 12 carcinoid tumors were typical carcinoids, and one (liver) was atypical carcinoid. Organoid pattern was present in 12 cases. Trabecular arrangement, ribbon arrangement, rosette formation, and pseudoglandular arrangement were recognized in 12 cases, in 8 cases, in 7 cases, and in 5 cases, respectively. Immunohistochemically, tumor cells were positive for at least one of pan-neuroendocrine markers including chromogranin, synaptophysin, neuron-specific enolase, CD56, and glucagon. Of these, synaptophysin was positive in 11/13 (85%), neuron-specific enolase 10/13 (80%), chromogranin 8/13 (62%), CD56 6/13 (46%), and glucagon 4/13 (31%). In summary, the author reported the incidence of digestive organ carcinoid tumors, and the clinicopathologic features of the 13 cases with carcinoid.
doi:10.4021/gr2009.01.1268
PMCID: PMC5139883  PMID: 27956948
Digestive organs; Carcinoid; Clinicopathology; Immunohistochemistry
8.  Primary pulmonary carcinoid tumor with metastasis to endometrial polyp 
INTRODUCTION
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.
PRESENTATION OF CASE
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.
DISCUSSION
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.
CONCLUSION
This is the first case in the literature described a malignant carcinoid metastasis to an endometrial polyp.
doi:10.1016/j.ijscr.2012.10.007
PMCID: PMC3537946  PMID: 23127865
Metastatic carcinoid tumor; Endometrial polyp
9.  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.
doi:10.3748/wjg.v11.i24.3794
PMCID: PMC4316038  PMID: 15968742
Carcinoid tumor; Accessory papilla; Polycythemia vera
10.  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.
doi:10.3978/j.issn.2072-1439.2012.s005
PMCID: PMC3537397  PMID: 23304441
Carcinoid; carcinoid tumor/complications; paraneoplastic neurological syndrome; paraneoplastic syndromes/etiology; polyneuropathy; paraparesis
11.  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.
doi:10.3748/wjg.v17.i35.4052
PMCID: PMC3199567  PMID: 22046097
Gastric carcinoid; Antrectomy; Endoscopic resection; Hypergastrinemia; Iron deficiency anemia
12.  Primary Neuroendocrine Tumor (Carcinoid Tumor) of the Testis: A Case Report with Review of Literature 
Patient: Male, 34
Final Diagnosis: Primary neuroendocrine tumor of the testis
Symptoms: Pain • swelling
Medication: None
Clinical Procedure: Radical orchiectomy
Specialty: Oncology
Objective:
Rare disease
Background:
The term carcinoid (Karzinoide) was coined by German pathologist Oberndorfer in 1907. Primary testicular carcinoid tumors (TCT) are rare, constituting 0.23% of all testicular tumors. In this report we describe a case of primary TCT of the testis and present the results of an extensive literature review to cover all the aspects of carcinoid tumor, including the definition, classification, origin, presentation, diagnostic evaluation, management, prognosis, and follow-up.
Case Report:
A 34-year-old male presented with chronic right scrotal swelling with recent onset of pain. Radical orchiectomy revealed a solid intratesticular tumor confined to the testis and epididymis, without lymphovascular invasion. Histology was consistent with neuroendocrine carcinoma. The tumor was staged as pT1 N0 M0 S2. Immunohistochemistry was positive for neuroendocrine markers. An extratesticular carcinoid tumor was ruled out. Urinary excretion of 5-hydroxyindoleacetic acid and Chromogranin A were within normal range.
Conclusions:
It is important to follow serotonin levels since the elevated levels of serotonin can cause carcinoid heart disease. If metastatic lesions are not accessible for resection, a trial of octreotide therapy can be given. This case also adds to the rare reports in the literature of primary carcinoid tumors of the testis having low malignant potential. The literature review highlights new diagnostic and therapeutic interventions and stresses the importance of long-term follow-up due to evidence of delayed metastasis or recurrences and also due to emergence of new complications as a result of improved prognosis and prolonged survival.
doi:10.12659/AJCR.894463
PMCID: PMC4463997  PMID: 26027014
Carcinoid Heart Disease; Carcinoid Tumor; Hydroxyindoleacetic Acid; Orchiectomy
13.  Carcinoid Syndrome-Induced Ventricular Tachycardia 
Case Reports in Cardiology  2016;2016:9142598.
Introduction. Carcinoid tumors are rare neuroendocrine malignancies that secrete multiple bioactive substances. These bioactive substances are responsible for the carcinoid syndrome characterized by diarrhea, flushing, syncope, and right-sided valvular heart disease. Previous case reports have described carcinoid syndrome associated with coronary vasospasm and the well-characterized carcinoid heart disease. Case. Our patient is a 73-year-old female with complex past medical history most notable for metastatic carcinoid tumors diagnosed in 2013-05. She initially presented in 2014-09 with syncope and dizziness associated with sinus pause on an event monitor. She received a pacemaker given normal left ventricular function and was discharged. However, she was readmitted with similar symptoms corresponding to multiple episodes of ventricular tachycardia. She was started on high-dose beta blockade and has had no recurrence of arrhythmia over a follow-up period of 12 months. Conclusion. We hypothesize that the patient's ventricular tachycardia was mediated by the multiple bioactive substances secreted by her carcinoid tumors. Her carcinoid tumor biomarkers were elevated and other explanations for arrhythmia were investigated and ruled out. To our knowledge, this is the first case of ventricular tachycardia mediated by carcinoid syndrome and suppressed by beta-blocker. Further investigation into this relationship is needed.
doi:10.1155/2016/9142598
PMCID: PMC4819085  PMID: 27088017
14.  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.
Background
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.
Methods
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.
Results
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.
Conclusion
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.
doi:10.1186/1746-1596-4-17
PMCID: PMC2704177  PMID: 19523243
15.  Clinicopathological characteristics and prognosis of resected cases of carcinoid tumors of the lung 
Thoracic Cancer  2016;7(6):633-638.
Background
Lung carcinoid tumors are rare, low‐grade, malignant neoplasms with some unclear features. The aim of this study was to analyze clinicopathological features and long‐term survival in patients with primary lung carcinoid tumors.
Methods
Patients who underwent surgery in our clinic and were diagnosed with carcinoid tumors, between August 1997 and July 2012, were included in the study. Patient characteristics and clinicopathological factors were retrospectively evaluated.
Results
Bronchial carcinoids comprised only 1.0% of lung cancer cases treated by resection. They are classified into two distinct categories: typical carcinoid (TC) and atypical carcinoid (AC) tumors. AC tumors occurred more frequently in younger patients and in smokers, and had a poorer prognosis than TC tumors. Overall three and five‐year survival rates for TC and AC were 92.6% and 81.1%, respectively. Univariate analysis showed that tumor size (P = 0.012) and histological type (P = 0.013) are prognostic factors. Multivariate analysis revealed that only tumor size (P = 0.044) was an independent prognostic factor.
Conclusions
The prognosis for bronchial carcinoid tumors was better than other types of lung cancer and TC was significantly better than AC. Radical lymph node dissection was the best treatment, with complete removal of the tumor. Tumor size was an independent prognostic factor for bronchial carcinoid tumors.
doi:10.1111/1759-7714.12377
PMCID: PMC5093170  PMID: 27755793
Carcinoid; lung cancer; surgery
16.  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.
doi:10.1155/2013/579396
PMCID: PMC3755439  PMID: 23997766
17.  Prognosis and risk factors of metastasis in colorectal carcinoids: results of a nationwide registry over 15 years 
Gut  2007;56(6):863-868.
Background
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.
Aim
To determine the predictive factors for metastasis in colorectal carcinoids and clarify their prognosis compared with adenocarcinomas.
Methods
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.
Results
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.
Conclusions
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.
doi:10.1136/gut.2006.109157
PMCID: PMC1954860  PMID: 17213340
18.  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.
Background
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.
Methods
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.
Results
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).
Conclusion
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.
doi:10.1186/1746-1596-2-15
PMCID: PMC1884130  PMID: 17509135
19.  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
20.  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
21.  Analysis of the molecular features of rectal carcinoid tumors to identify new biomarkers that predict biological malignancy 
Oncotarget  2015;6(26):22114-22125.
Although gastrointestinal carcinoid tumors are relatively rare in the digestive tract, a quarter of them are present in the rectum. In the absence of specific tumor biomarkers, lymphatic or vascular invasion is generally used to predict the risk of lymph node metastasis. We, therefore, examined the genetic and epigenetic alterations potentially associated with lymphovascular invasion among 56 patients with rectal carcinoid tumors. We also conducted a microRNA (miRNA) array analysis. Our analysis failed to detect mutations in BRAF, KRAS, NRAS, or PIK3CA or any microsatellite instability (MSI); however, we did observe CpG island methylator phenotype (CIMP) positivity in 13% (7/56) of the carcinoid tumors. The CIMP-positive status was significantly correlated with lymphovascular invasion (P = 0.036). The array analysis revealed that microRNA-885 (miR-885)-5p was the most up-regulated miRNA in the carcinoid tumors with lymphovascular invasion compared with that in those without invasion. In addition, high miR-885-5p expression was independently associated with lymphovascular invasion (P = 0.0002). In conclusion, our findings suggest that miR-885-5p and CIMP status may be useful biomarkers for predicting biological malignancy in patients with rectal carcinoid tumors.
PMCID: PMC4673150  PMID: 26090613
carcinoid; non-coding RNA; epigenetics; neuroendocrine tumor; rectum
22.  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.
doi:10.3892/ol.2012.622
PMCID: PMC3389625  PMID: 22783400
carcinoid tumor; skeletal metastasis; prognosis
23.  Metastatic Gastrointestinal Carcinoid Tumor with Unknown Primary Site 
Radiology Case Reports  2015;2(3):90.
Carcinoid tumors are rare and slow growing malignancies derived from enterochromaffin cells. Two-thirds of carcinoid tumors arise in the gastrointestinal tract, and in 3% of these cases the primary site cannot be determined. Presenting symptoms depend on the location of the primary tumor but may be nonspecific, and in 13% of patients distant metastases are discovered on diagnosis. The classic carcinoid syndrome occurs in less than 10% of cases and only after metastasis to the liver. We present a case of a young woman with a gastrointestinal carcinoid tumor of unknown site that had metastasized to the liver. We also provide a review of the current diagnostic modalities. Familiarity with the signs and symptoms of carcinoid tumors and the diagnostic techniques thereof may facilitate early detection and improved outcome.
doi:10.2484/rcr.v2i3.90
PMCID: PMC4895068  PMID: 27303478
CT, computed tomography
24.  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
25.  Renal carcinoid tumor with liver metastasis followed up postoperatively for 9 years 
Diagnostic Pathology  2015;10:182.
Background
We describe a case of renal carcinoid tumor with liver metastasis followed up postoperatively for 9 years.
Case presentation
A 33-year-old man presented with left flank dull ache. On the abdominal computed tomography, a solid renal mass in the upper portion of the left kidney was detected. The patient had no other abnormal findings, such as suspected distant metastasis or lymph node metastasis. Radical nephrectomy was performed on 14/9/2005. Histological examination and immunohistochemical staining confirm primary renal carcinoid tumor. 9 years after radical nephrectomy, computed tomography of the abdomen demonstrated a 2 cm × 1.8 cm cyst mass in the right liver. Similar pathologic characteristics were found between the renal carcinoid tumor and liver tumor.
Conclusions
We present a primary renal carcinoid tumor with liver metastasis 9 years after radical nephrectomy. With literature review, renal carcinoid tumors exhibit heterogenous behavior.
doi:10.1186/s13000-015-0417-7
PMCID: PMC4596506  PMID: 26445413
Kidney; Carcinoid; Metastasis

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