Introduction. Carcinoid tumors are low-grade tumors originating from endoderm and mostly involving the gastrointestinal system. However; they may be seen in any site within the gastrointestinal system. Case Presentation. A 69-year-old female patient. The results of blood tests were observed to be consistent with obstructive jaundice. A mass appearance was not encountered on tomographic examination. Papilla that was tumor-like macroscopically was seen in the second part of the duodenum in diagnostic endoscopy. Pylorus—preserving pancreaticoduodenectomy surgical procedure was applied. On pathological examination of the mass, a tumoral mass was detected in ampulla vateri localization, 1.5 × 1 × 0.8 cm in size, which, in immunohistochemical staining, was evaluated as a neuroendocrine tumor. Also, Metastasis was observed. Conclusion. The rarest type of carcinoid tumor is ampullary located carcinoid tumor, and tumor size is not a reliable indicator for tumor aggressivity in ampullary carcinoid tumors.
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.
Gastric carcinoid; Antrectomy; Endoscopic resection; Hypergastrinemia; Iron deficiency anemia
Pulmonary carcinoids are infrequent neoplasms of the lung that normally display a less aggressive biological behavior compared to small cell and non-small cell lung cancers. Approximately 15-25% of carcinoids, in particular atypical carcinoids, show lymph node metastasis and have a worse prognosis than their non-metastasized counterparts. To date, there is no morphological or molecular marker that may help to differentiate between carcinoids that metastasize and carcinoids of identical differentiation that show only local tumor growth. In this study, we analyzed 7 metastasized and 10 non-metastasized pulmonary carcinoids for chromosomal and microsatellite instability in order to determine whether microsatellite instability or chromosomal imbalances are associated with metastasis. Due to the rare occurrence of metastasized carcinoids we compared our results of chromosomal instability with the hitherto published comparative genomic hybridization (CGH) profiles of pulmonary carcinoids, for which information about the absence or presence of metastasis was available. While microsatellite instability was not detected we found chromosomal instability as a common event in pulmonary carcinoids with an increase of frequency and extent of chromosomal alterations in atypical and metastasized carcinoids. These findings are in accordance with the collected and herein compiled data of previous studies and indicate increasing numbers of chromosomal imbalances to play a role in the sequential process of tumor development and metastasis.
biological markers; carcinoid tumor; comparative genomic hybridization; lung neoplasms; microsatellite instability; neoplasm metastasis
Carcinoid is an endocrine cell tumor with low-grade atypia, which is generally a low-grade malignant cancer with a good prognosis. Metastatic renal carcinoid is even rarer than primary carcinoids.
We present our experience of a patient with metastatic renal carcinoid from the gastrointestinal tract.
The carcinoid tumor of the kidney in our patient, who had a history of liver metastasis from rectal carcinoid, was considered metastatic based on the pathological findings.
Carcinoid tumors are diminutive growths found along the gastrointestinal tract, in the lungs and, occasionally, within the confines of teratomas. Carcinoid tumors of the intestinal tract are found in decreasing order of frequency in the rectum, the appendix, the ileum, the jejunum, the lung, the stomach and the duodenum. Arising from argentaffin elements in the crypts of Lieberkuehn, and secreting serotonin, carcinoid tumors belong to the order of functioning neoplasms. Their indolent growth seems to place them between benign neoplasms and carcinomas. However, they possess propensities for metastasis that correlate with increase in size.
The symptoms that are produced are almost always due to the presence of metastatic lesions. In ileal and gastric carcinoid tumors, the tendency to metastasis is augmented and the metastatic masses are sometimes of sufficient bulk to cause the carcinoid syndrome or to interfere with the supply of blood to the affected segment. More often they produce the clinical picture of intestinal obstruction.
The unusually long interval from onset to death associated with carcinoid tumors makes palliative subtotal resections and short-circuiting operations in symptomatic patients with advanced disease worthwhile, for by such unorthodox procedures the patients may be afforded many additional years of useful life.
A gastric carcinoid tumor concomitant with gastrointestinal stromal tumor (GIST) is rarely encountered in clinical practice. We report a 65-year-old female who had a 0.8 cm gastric carcinoid tumor on the posterior wall of the upper gastric corpus detected during an esophagogastroduodenoscopy at a routine physical examination, and a concomitant 1.1 cm GIST on the anterior wall of the upper gastric corpus incidentally found during surgery of the gastric carcinoid tumor. Normal serum gastrin level and histological findings suggested that she had a type III gastric carcinoid tumor and a GIST which were categorized a very low risk of malignancy, based on their small size and lack of mitosis. Both tumors were treated successfully by surgical excision. The patient had an uneventful recovery. Neither recurrence nor metastasis was found after a 28-mo follow-up.
Gastric carcinoid tumor; Gastrointestinal stromal tumor; Esophagogastroduodenoscopy; Digestive system
We report a case with features of gastric adenocarcinoma colliding with a typical carcinoid component. A 51-year-old female was admitted to the Department of Internal Medicine with complaints of epigastric pain. On physical examination of the patient there was significant epigastric tenderness and the CA19-9 level was higher than the normal titer value. An upper gastrointestinal endoscopy showed an ulcerated polypoid mass located on the cardiac region of the stomach. Pathological and immunohistochemical findings diagnosed as a collision tumor comprising both adenocarcinoma and carcinoid tumor. Metastasis of adenocarcinoma was found in 7 perigastric lymph nodes, while metastasis of the carcinoid was not detected. The admixture of neoplastic endocrine and nonendocrine cells, have been found infrequently in gastric tumors. The mixed tumors can be further classified into composite tumors that show an admixture of two histological components with histological transitions and collision tumors where the two components are not intermixed In general it is not easy to morphologically distinguish a collision tumor, from composite tumor. Microscopically, hematoxylin and eosin-stained tissue sections from two different areas of the mass revealed two different types of tumor; an intestinal type adenocarcinoma and a carcinoid tumor. We report a case with features of adenocarcinoma colliding with a typical carcinoid component, along with a review of the literature.
collision tumor; gastric adenocarcinoma; carcinoid
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.
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.
Metastatic carcinoid tumor; Endometrial polyp
Carcinoid and neuroendocrine tumors of the colon and rectum arise from the amine precursor uptake and decarboxylation (APUD) cells of the intestine. Carcinoid tumors are most commonly found in the gastrointestinal tract and are located in decreasing order of frequency in appendix, ileum, rectum, stomach, and colon. The vast majority of lesions are asymptomatic and are found incidentally during endoscopy. The management of these lesions depends upon the size of the lesion, involvement of the muscularis, location, and presence of metastatic disease. Small lesions (1 cm) can often be treated locally, either endoscopically or transanally. However, larger lesions (> 2 cm) require a formal oncologic resection. Adjuvant therapy is indicated only for metastatic disease, and admirable advances have been made in the realm of chemotherapy for reduction of disease and palliation of the symptoms of carcinoid syndrome. In this article, we discuss the nature of these interesting and uncommon tumors, clinical presentation, treatment options, and prognosis.
Carcinoid; neuroendocrine tumor; colon; rectum
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.
Carcinoid; carcinoid tumor/complications; paraneoplastic neurological syndrome; paraneoplastic syndromes/etiology; polyneuropathy; paraparesis
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.
carcinoid tumor; skeletal metastasis; prognosis
We report 3 cases of gastric carcinoids with hypergastrinemia. Case 1: A 60-year-old man had a 2 cm carcinoid of the stomach and underwent partial resection. Involvement of the muscularis propria and lymph nodes metastasis were observed microscopically. Follow-up gastroscopy revealed another carcinoid lesion and total gastrectomy was performed. Case 2: A 67-year-old woman with multiple carcinoids of the entire stomach underwent antrectomy. No growth of residual tumors has been detected so far. Case 3: A 61-year-old man had a tumor near the esophagogastric junction and underwent total gastrectomy. Carcinoid component was diffusely intermingled with adenocarcinoma in the tumor and invaded into the subserosa. In all 3 cases, the serum gastrin level was high and atrophic gastritis was microscopically observed. Carcinoid tumor in Case 3 was different from those in Cases 1 and 2 and interestingly, gastric carcinoid with hypergastrinemia showed various types of appearance.
Endoscopic submucosal dissection (ESD) for upper gastrointestinal (GI) subepithelial tumors (SETs) originating in the muscularis propria (MP) layer is associated with numerous issues regarding secure closure and measures against accidental perforation. However, symptomatic benign GI SETs or carcinoid tumors originating in the submucosa (SM) may be safely resected en-bloc using ESD. In this study, the feasibility and safety of ESD as a novel method for endoscopic resection for such GI SETs revealed on endoscopic ultrasonography (EUS) was investigated. A total of 12 consecutive cases of patients with symptomatic benign SETs (n=3; 1 esophageal hemangioma and 2 gastric lipomas) or small carcinoid tumors (n=9; <10 mm, with an extremely low risk of metastasis) originating in the SM as determined on EUS, between March, 2009 and April, 2013, were retrospectively reviewed. The lesions were resected by ESD after confirming that the tumors originated from the SM. The complication rate following en-bloc resection was also determined. En-bloc resection was achieved in all 12 cases, the mean procedure time was 45 min (range, 20–120 min) and no complications occurred intra- or postoperatively. There was no tumor recurrence or disease-related mortality reported during the follow-up period (median follow-up time, 13.4 months). Histopathological curative resection was achieved with ESD without complications in all 9 cases with carcinoid tumors. Therefore, if EUS reveals a SET originating in the SM without infiltration of the MP and resection is indicated due to the presence of abdominal symptoms, ESD may be a feasible option for diagnostic treatment with minimal invasiveness. However, larger-scale prospective studies are required to establish the feasibility and safety of this procedure.
endoscopic submucosal dissection; gastrointestinal subepithelial tumors; symptomatic benign tumors; small carcinoid tumors
It is often difficult to evaluate the grade of malignancy and choose an appropriate treatment for colorectal carcinoids in clinical settings. Although tumor size and depth of invasion are evidently not enough to stratify the risk of this rare tumor, the present guidelines or staging systems do not mention other clinicopathological variables. Recent studies, however, have shed light on the impact of lymphovascular invasion on the outcome of colorectal carcinoids. It has been revealed that the presence of lymphovascular invasion was among the strongest risk factors for metastasis along with tumor size and depth of invasion. Furthermore, tumors smaller than 1 cm, within submucosal invasion and without lymphovascular invasion, carry minimal risk for metastasis with 100% 5-year survival in the studies from Japan as well as from the USA. This would suggest that these tumors could be curatively treated by endoscopic resection or transanal local excision. On the other hand, colorectal carcinoids with either lymphovascular invasion or tumor size larger than 1 cm carry the risk for metastasis equivalent to adenocarcinomas. Therefore, it should be emphasized that histological examination of lymphovascular invasion is mandatory in the specimens obtained by endoscopic resection or transanal local excision, as this would provide useful information for determining the need for additional radical surgery with regional lymph node dissection. Although the present guidelines or TNM staging system do not mention the impact of lymphovascular invasion, this would be among the next promising targets in order to establish better guidelines and staging systems, particularly in early-stage colorectal carcinoids.
Lymphovascular invasion; Neuroendocrine tumor; Carcinoid; Colorectal cancer
Carcinoids are neuroendocrine tumors (NETs) that secrete hormones, including serotonin, resulting in the malignant carcinoid syndrome. In addition to the significant morbidity associated with the syndrome, carcinoids are frequently metastatic at diagnosis and untreated mortality at 5 years tops 70%. Surgery is the only curative option and the need for other therapies is clear. We have previously shown that activation of Raf-1 inhibits carcinoid cell proliferation.
We investigated the ability of Leflunomide (LFN), an FDA approved medication for the treatment of rheumatoid arthritis, and its active metabolite Teriflunomide (TFN) as a potential anti-NET treatment. LFN and TFN inhibit the in vitro proliferation of gastrointestinal carcinoid cells and induce G2/M phase arrest. Daily oral gavage of nude mice with subcutaneous xenografted carcinoid tumors confirms that LFN can inhibit NET growth in vivo. Treatment with TFN suppresses the cellular levels of serotonin and chromogranin A, a glycopeptide co-secreted with bioactive hormones. Additionally TFN reduces the level of Achaete-Scute Complex-Like 1 (ASCL1), a NET marker correlated with survival. These effects are associated with the activation of the Raf-1/MEK/ERK1/2 pathway and blockade of MEK signaling reversed the effects of TFN on markers of the cell cycle and ASCL1 expression.
In summary, LFN and TFN inhibit carcinoid cell proliferation in vitro and in vivo and alter the expression of NET markers. This compound thus represents an attractive target for further clinical investigation.
Leflunomide; Teriflunomide; Carcinoid; Raf-1; Achaete-Scute Complex-Like 1
To report the clinical and radiographic features and treatment outcome of neuroendocrine tumor (carcinoid) metastasis to the orbit.
Materials and Methods:
Retrospective chart review of four cases.
Mean patient age at the time of diagnosis of the primary neuroendocrine tumor and orbital metastasis was 58 and 66 years, respectively, with a mean duration of 8 years between diagnosis of primary tumor and orbital metastasis. Primary neuroendocrine tumor sites were gastrointestinal tract (n = 2), lung (n = 1), and testicle (n = 1). The most common presenting symptom was diplopia (three cases). Magnetic resonance imaging revealed orbital tumor in all cases. Octreotide scan was positive in one case. Treatment was tumor excision in three cases followed by external beam radiotherapy in two cases and one patient was followed without treatment. Tumor cells showed immunoreactivity to chromogranin, synaptophysin, and neuron-specific enolase in all cases. Mean follow-up after orbital tumor diagnosis was 39 months. Three patients had known systemic extraorbital metastasis before orbital involvement (mean interval of 5.9 years) and one case had immediately after development of orbital metastasis. One patient had multiple recurrences of orbital metastasis and eventually underwent exenteration. Two patients died of disseminated metastasis between 2 and 3 years after diagnosis of orbital metastasis.
All four patients with orbital metastasis from neuroendocine tumor had evidence of systemic extraorbital metastasis. Aggressive metastatic neuroendocine tumors of orbit can lead to local recurrence even after surgical excision and radiation. Imaging tests were helpful in allowing early diagnosis and for monitoring after treatment.
Carcinoid tumor; magnetic resonance imaging; metastasis; neuroendocrine tumor; octreoscan
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.
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.
The current study presents the case of a patient with a recurrent carcinoid tumor of the ovary, 13-years after the primary surgery. The primary surgery consisted of a total abdominal hysterectomy and bilateral salpingo-oophorectomy for a left ovarian tumor at 54 years old. Pathologically, the tumor was diagnosed as a carcinoid tumor of the ovary. Following the primary treatment, the patient was admitted to a cardiologist due to carcinoid-induced heart failure. At 67 years old, the patient was referred to Kyoto University Hospital with a solitary mass 8 cm in diameter and located in the paraaortic area, which was detected by routine ultrasonography and subsequent computed tomography (CT) scans. Urinary 5-hydroxyindole acetate (5-HIAA), a serotonin degradation metabolite, was present at elevated levels. With a diagnosis of a recurrent carcinoid tumor, the patient underwent a tumor resection. The pathological diagnosis was that of lymph node metastasis of the trabecular carcinoid. Post-operatively, the 5-HIAA levels returned to normal. Carcinoid tumors occasionally recur following surgery due to borderline malignant potential. Due to the slow growing nature of these tumors, in specific cases, recurrence occurs following a long interval. Therefore, a relatively long follow-up period is required.
carcinoid of the ovary; recurrence
A 51-year-old woman with severe asthma underwent bronchoscopy and endobronchial ultrasound (EBUS) for investigation of a 15-mm peripheral lung nodule. Histology demonstrated a typical carcinoid tumor. Pulmonary location is the second commonest site for carcinoid tumors. Diagnosis of peripheral carcinoid tumor of the lung is difficult due to its small size, poor accuracy of cytologic diagnosis, and low sensitivity of positron emission tomography in detecting it. EBUS has a high diagnostic yield and a low complication rate in the evaluation of small solitary pulmonary nodules. The ultrasound appearance of carcinoid tumors is identical to that of lung carcinomas. Prompt diagnosis of carcinoid tumor is desirable as regional lymph node metastasis is seen in 10% of patients and is associated with a reduced 5-year survival. We feel that, where possible, all patients presenting with solitary pulmonary nodules should be investigated initially using EBUS due to its high diagnostic rate and the very low incidence of adverse events.
Pulmonary carcinoid tumor; endobronchial ultrasound; solitary pulmonary nodule
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.
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.
Gastrointestinal stromal tumor is the most common mesenchymal tumor in the gastrointestinal tract. It may coexist with other type of cancers, and if so, the tumors usually involve the stomach. The most common associated cancers are gastrointestinal carcinomas. We report a 65-year-old woman with a history of gastric gastrointestinal stromal tumor who had undergone subtotal segmental gastrectomy. New polypoid lesions were detected on a follow-up gastroscopy one year later. The lesions were biopsied and found to be carcinoid tumors. There was serum hypergastrinemia, and type 1 gastric carcinoid tumor was diagnosed. A total gastrectomy was performed. Pathologic examination revealed both carcinoid tumors and a recurrent gastrointestinal stromal tumor.
Hypergastrinemia; Multiple primary neoplasms; Stomach; Gastrointestinal stromal tumor; Carcinoid tumor
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.
Carcinoid; Primary hepatic carcinoid; Neuroendocrine neoplasm; Therapy; Surgical treatment; Prognosis
Monodermal teratomas of the ovary can take the form of carcinoid tumors of which there are several types, mucinous carcinoid being the least common. Very few cases of primary mucinous carcinoid of the ovary have been reported in the literature and the behavior of these tumors over the long term is unclear. We describe a case of primary mucinous carcinoid of the ovary in a 39-year-old woman treated with unilateral salpingo-oophorectomy, where a metastasis occurred in the contralateral ovary ten years later. This case demonstrates that mucinous carcinoid of the ovary can metastasize even after a long interval, and careful follow-up of patients, particularly those treated conservatively, is appropriate.
mucinous carcinoid; metastasis; ovary
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.
A 66 year old female was found to have an ampullary mass on endoscopic examination. A pancreaticoduodenectomy was performed and the pathology was consistent with carcinoid tumor. Carcinoid tumors of the ampulla of Vater are rare tumors of the gastrointestinal tract, accounting for 0.3-1% of gastrointestinal carcinoids. Management of ampullary carcinoid tumor with pancreaticoduodenectomy is in accordance with current recommendations.