Advanced endocrine cell carcinoma of the gallbladder: a patient with 12-year survival

Gallbladder carcinoid is a rare disease. In previous reports, classical carcinoid, an entity with a good prognosis, has not been distinguished from endocrine cell carcinoma, a tumor associated with marked cell atypia and mitosis, and a poor prognosis. The patient was a 66 year old woman who presented to our hospital with a chief complaint of jaundice. Pre-operatively, she was diagnosed as having advanced gallbladder carcinoma invading the liver and the hepatic hilus. The patient underwent right hepatic trisegmentectomy with en bloc resection of the caudate lobe and extrahepatic bile ducts, extended lymph node clearance and left hepaticojejunostomy. Histopathological examination showed positive Grimelius staining, marked mitosis, and intense atypism, hence, the tumor was diagnosed as an endocrine cell carcinoma. Twelve years after surgery, the patient is healthy, without any sign of recurrence. We present this novel case of long-term survival and review the literature.     

Carcinoid somatostatinoma of the papilla of Vater: a case report

A 57 year-old Japanese man with a carcinoid somatostatinoma of the papilla of Vater is presented. He was found to have cholecystolithiasis without any symptoms. Physical examination showed no abnormal findings. Routine laboratory data gave normal results, except for glucose intolerance and an elevated somatostatin concentration. A yellowish papillary tumor was found at the papilla of Vater, and histological examination suggested the diagnosis of carcinoid. He underwent a pancreatoduodenectomy in March 1992. The gallbladder contained a single pure cholesterol stone. Histological, immunohistochemical, and electron microscopic studies resulted in the diagnosis of a carcinoid somatostatinoma of the papilla of Vater, without regional lymph node metastases. Post-operative pancreatic juice output from the total pancreatic duct drainage increased to more than 1000 mL/day. Although an anastomotic leakage of the pancreatojejunostomy was noted, the pancreatic fistula closed 8 weeks later. His postoperative somatostatin value was normal. He has been well for 54 months following surgery, without any signs of recurrence.     

A cytogenetic deletion, del(17)(q11.22q21.1), in a patient with sporadic neurofibromatosis type 1 (NF1) associated with dysmorphism and developmental delay

Pure primary ovarian carcinoid tumors are uncommon and only 21 cases have been recorded in the literature. In the past 15 years, we have seen two cases. One was a strumal carcinoid and the other, the case presented here, was a primary ovarian carcinoid tumor arising from the left ovary of a 25-year-old woman who had no carcinoid syndrome. The tumor was made up of pure carcinoid tumor without other teratomatous elements. On light microscopy the neoplasm, composed of uniform tumor cells, was arranged in solid nests or a trabecular pattern. The differential diagnosis included granulosa cell tumor. However, the strongly argyrophilic, chromogranin staining and ultrastructural presence of neurosecretory granules confirmed the diagnosis of primary ovarian carcinoid tumor. After a careful survey of the contralateral ovary and the gastrointestinal tract, the patient underwent a left oophorectomy. Her postoperative course was uneventful. The literature and the pathologic findings are reviewed and discussed, along with the differential diagnosis and treatment of primary ovarian carcinoid tumor.     

Horseshoe kidney is associated with an increased relative risk of primary renal carcinoid tumor

PURPOSE: Carcinoid tumor is a rare neoplasm of the kidney with an unknown histogenesis. Of only 31 cases previously reported in the literature 4 arose within horseshoe kidneys. We report a case of primary carcinoid tumor arising within a horseshoe kidney and discuss the unique insight it provided into the pathogenesis of this tumor. MATERIALS AND METHODS: We reviewed in detail all 31 reported cases of renal carcinoid tumor and, using reported incidence rates of horseshoe kidney, we calculated the relative risk of renal carcinoid tumor arising within a horseshoe kidney. Immunohistochemical staining for neuroendocrine related markers was performed on tissue sections from the present carcinoid tumor, the adjacent kidney and 5 control samples of normal renal parenchyma. RESULTS: Of the reported tumors 15.6% occurred in horseshoe kidneys, yielding a calculated relative risk of 62. The present tumor was multifocal, arising from the wall of a cystic lesion and possibly representing a dilated calix within the isthmus. Intestinal epithelium lining the cyst cavity exhibited multifocal neuroendocrine cell hyperplasia with an immunohistochemical profile identical to that of the carcinoid tumor cells. CONCLUSIONS: The relative risk of renal carcinoid tumor developing in a horseshoe kidney is markedly greater than that for Wilms tumor or transitional cell carcinoma. The clinical course of renal carcinoid tumor arising within a horseshoe kidney appears to be more benign than that of the nonhorseshoe variant. Our observations support the hypothesis that renal carcinoid tumors may arise from neuroendocrine cells within foci of metaplastic or teratomatous epithelium within the kidney.     

Carcinoid of the gallbladder. A report of a clinical case treated by video laparoscopic cholecystectomy

The authors report a clinical case of the gallbladder carcinoid tumour treated with laparoscopic cholecystectomy. After a short analysis of the classification, the malignant potentiality and the symptoms of gallbladder carcinoids, they conclude that it is possible to treat carcinoids with laparoscopic cholecystectomy, but only in some selected cases, that is to say in the absence of factors indicative of local invasion and that in the other cases it is preferable the classic surgical treatment of laparotomic resection.     

Carcinoid tumor of the thymus with divergent sarcomatoid differentiation: report of a case with histogenetic consideration

An anterior mediastinal tumor resected from a 54-year-old man without paraneoplastic symptoms was found to be an unusual biphasic thymic tumor composed of carcinoid tumor in sarcomatous stroma characterized by fibrosarcoma-like spindle cells with areas of chondroid and osseous differentiation. Immunohistochemical and ultrastructural studies verified that the tumor described was a carcinoid tumor with divergent sarcomatoid differentiation rather than a mixed carcinoid tumor and sarcoma. The chondroid and osseous differentiations observed were unique features. This tumor is rare and provides an opportunity for understanding tumors with divergent components. The sarcomatoid transformation of thymic carcinoid tumor might denote highly malignant clinical behavior as illustrated by this case.     

Carcinoid tumor metastatic to the breast

Breast metastases from nonmammary malignant neoplasms are uncommon, accounting for approximately 2% of breast tumors. There are 13 cases reported in the literature of carcinoid tumor metastatic to the breast, and more than half of these cases were misdiagnosed pathologically and treated as primary breast carcinoma, even in cases with a medical record of carcinoid tumor. We describe a patient with a history of asthma and diarrhea who presented to the University of Arkansas for Medical Sciences, Little Rock, with an exacerbation of the asthma. The results of routine physical examination revealed a mass in the left breast. A diagnosis of carcinoid tumor metastatic to the breast was made after a partial mastectomy was performed. The differential diagnosis between primary carcinoid tumor of the breast and carcinoid tumor metastatic to the breast is often controversial in surgical pathology. Diagnoses need to be made correlating clinical and histological examination in difficult cases in which there is not a diagnosis of carcinoid tumor elsewhere. Accurate diagnosis of breast metastases is important to avoid unnecessary treatment.     

Failure to control the airway in a patient with Hunter''s syndrome

We report a 36-year-old man with proximal dominant muscle weakness, thymic tumor, diabetes mellitus, hypokalemia, and increased levels of plasma ACTH and cortisol. The diagnosis of carcinoid tumor was made on the basis of pathological findings in the biopsied specimen of the thymic tumor. The proximal muscle weakness was considered to be due to steroid myopathy resulting from the overproduction of cortisol from the adrenal glands induced by the ectopic ACTH secreted by the thymic carcinoid tumor. Although thymoma in frequently associated with myasthenia gravis, we should also consider carcinoid tumors in patients with a thymic tumor presenting with a proximal muscle weakness.     

Carcinoid tumor occurring in cystic teratoma of the kidney: a case report

Although carcinoid tumors have occurred rarely in benign teratomas of the ovary and the testis, we believe this case is the first reported of carcinoid arising in a benign cystic teratoma of the kidney. The patient was admitted to the Tagawa Hospital with a left abdominal mass and severe epigastric pain. Retrograde and intravenous pyelograms revealed marked dilatation of the left kidney; a renal tumor was diagnosed clinically. At operation, a polycystic tumor weighing 1400g was removed. Histologically, a carcinoid tumor was evident among teratoid tissues, such as columnar epithelium, cartilage, smooth muscle, and mucous secretory glands. The patient did not have a carcinoid syndrome and had an uneventful recovery.     

Primary hepatic carcinoid tumor

A case of primary carcinoid tumor arising in the liver of a 69 year old woman with no endocrine symptoms is reported. Histopathologically, the tumor was diagnosed initially as a hepatocellular carcinoma in the biopsy specimen, and was shown subsequently to be a carcinoid tumor, demonstrating diffuse positive staining with Grimelius method. Mucin stained with periodic acid-Schiff (PAS), alcian-blue, and mucicarmine, and was shown partially in the glandular structures. Immunohistochemically, most of the tumor cells stained positively for chromogranin-A, epithelial membrane antigen (EMA) and neuron specific enolase (NSE). Ultrastructural examination revealed electron-dense core granules, measuring 40-120 nm in diameter in some of the tumor cells. Intensive and careful searches pre- and post-operatively revealed no other primary source of tumor other than the liver. The patient was reported well with no symptoms 3 1/2 years after the operation. This case is considered to be a primary hepatic carcinoid tumor. The recent literature is reviewed, and the possible histogenesis of hepatic carcinoid tumor is discussed.     

Assessment of self-injurious behavior maintained by access to self-restraint materials

Metastatic tumor is one of several etiologies of space-occupying masses in the orbit that accounts for 1%-13% of all orbital masses (1). In the adult patient population, breast cancer is the most common tumor to metastasize to the orbit followed by metastases from the lung, prostate and gastrointestinal tract (2). It is rare for carcinoid tumors to metastasize to the eye or to the orbit. Carcinoid tumors arise from Kulchitsky cells that originate in the neural crest. Histologically, these tumors resemble, but are not as aggressive as, adenocarcinomas. Most carcinoids arise in the gastrointestinal tract or the lung. The most common site for carcinoid metastases is the liver. On anatomical imaging studies, such as CT and magnetic resonance imaging, metastatic orbital carcinoid tumors appear as nonspecific tumor masses. Carcinoid tumors have an affinity for uptake of the radiopharmaceutical 131I-metaiodobenzylguanidine (MIBG) (3). We report a case of a patient with a known carcinoid tumor who developed a left orbital mass that demonstrated abnormal uptake of 131I-MIBG indicative of metastatic carcinoid tumor to the orbit.     

Carcinoid tumor as a part of mature teratoma of the testis

A rare case of primary carcinoid tumor in otherwise mature teratoma of the testis which occurred in a 70-year-old male is reported. The patient was treated by unilateral orchiectomy. The pathological specimen showed morphological characteristics of carcinoid in the teratomatous tumor. The immunohistochemical studies revealed positivity for chromogranin and neuron-specific enolase. Grimelius staining showed argyrophil granules in cells. Symptoms of a carcinoid syndrome were not noted. The patient remains disease free after surgery.     

Perceived motivational climate and cognitive and affective correlates among Norwegian athletes

CONCLUSION: The diagnosis of a pancreatic carcinoid should be based on the measurement of serotonin in serum or its demonstration in the tumor and/or by the measurement of its derivative (5-HIAA) in urine. Carcinoid of the pancreas is a rare but definite entity; usually having metastasized by the time of diagnosis. The term "serotonin-producing tumor of the pancreas" has been suggested as an alternative designation for "pancreatic carcinoid." BACKGROUND: The literature on carcinoid tumors of the pancreas is confusing because much of it preceded the development of the more specific immunological, chemical and staining techniques currently available. METHODS: 43 case reports were collected from the world's literature, based on a demonstrable pancreatic neuroendocrine tumor plus a positive finding of at least one of the following without another dominant hormone being demonstrated: elevation of 5-Hydroxytryptamine (5-HT) (serotonin) in the serum or detected in tumor tissue, and/or elevation of 5-Hydroxyindole acetic acid (5-HIAA) in the urine. In addition to these two hormone-specific assays, information was collected on the silver-staining properties of the tumor; properties which have traditionally been associated with carcinoid tumors. Positive silver staining in tumor cells (argyrophilic and/or argentaffin reaction) is strongly indicative of the carcinoid tumor but the findings are less specific than the hormone assays and immunohistologic stains. RESULTS: In this review of 43 cases, including two current ones, the pancreatic carcinoid tumor has the following important features: 1. It is a rare tumor that is usually diagnosed late when the tumor is large and has metastasized. Thirty-eight (88.4%) have been malignant. They are, therefore, associated with a high incidence of the "carcinoid syndrome." 2. To date, prognosis in therapy is poor, based on delayed diagnosis, a resultant low incidence of resectability, and an uncertain duration of survival after resection. 3. Pancreatic carcinoid tumors remain difficult to differentiate from other endocrine tumors. The measurement of urinary 5-HIAA excretion or the demonstration of elevated serotonin level in the tumor or in serum is essential to its distinction. Silver staining of the tumor, although of historic importance, has been superceded by the hormone-specific studies. 4. To distinguish it from other endocrine tumors of the pancreas, the terms "pancreatic serotoninoma" or "serotonin-producing tumor of the pancreas" have been suggested as possible alternatives. Its growth characteristics may be related more to its cell of origin than to its extent of hormone secretion. Not all of the tumors result in recognizable hyperserotoninemia.     

Gastrin and gastrin receptor

Gastrin is a well known endogenous stimulator of gastric acid. In addition, recent studies have revealed that gastrin has a growth promoting effect on gastric ECL cells. Indeed, development of ECL carcinoid tumor occurs almost exclusively in patients with hypergastrinemia such as autoimmune gastritis and Zollinger-Ellison syndrome with MEN type I. We have recently cloned human gastrin receptor gene, and by using it, we found that both gastric carcinoid tumor and endocrine cell carcinoma of the stomach express significant amount of gastrin receptor gene whereas none of gastric cancer tissue shows gastrin receptor gene expression. Thus, it is clear that gastrin plays important roles in the development of gastric carcinoid tumor as well as endocrine cell carcinoma of the stomach.     

Tryptophan hydroxylase antibodies used in the diagnosis of carcinoid

The expression of tryptophan hydroxylase, the rate-limiting enzyme in the biosynthesis of serotonin, is described in a case of a 35 year-old patient with metastatic jejunal carcinoid. Immunohistochemically, monoclonal anti-tryptophan hydroxylase antibodies positively identified liver metastases of a neuroendocrine tumor. The cellular distribution of tryptophan hydroxylase was restricted exclusively to the cytoplasm of carcinoid cells, where it was found in large amounts. By means of immunoblotting, anti-tryptophan hydroxylase antibodies detected in samples from carcinoid tissue two closely migrating polypeptide bands with molecular weights of 26 kDa and 29 kDa, respectively. These two protein bands appear to represent proteolytically degraded polypeptides, since tryptophan hydroxylase is known for its extreme unstability in vitro. In our case, the immunohistochemical and biochemical identification of tryptophan hydroxylase in liver lesions of a neuroendocrine tumor permitted the correct diagnosis of a metastatic carcinoid.     

Malignant carcinoid tumor and synchronous malignant extraadrenal paraganglioloma

Gastrointestinal carcinoid tumor is often considered the most common neuroendocrine tumor of the small intestine. The overall incidence of 1% in the general population is quite low. Extraadrenal paragangliomas are rarer still, and the incidence of both of these tumors in the malignant state is exceedingly rare. This article describes the case of a patient who had both a malignant carcinoid tumor as well as a malignant retroperitoneal paraganglioma occurring synchronously. A review of the literature concerning these tumors is also presented.     

A case of malignant strumal carcinoid

A malignant strumal carcinoid with widespread metastases resembling a well-differentiated thyroid follicular carcinoma is described. Strumal carcinoid is a rare ovarian tumor which usually behaves in a benign manner. Only one malignant case has been reported in the literature and in that case the carcinoid element metastasized. The clinical, histological, and immunohistochemical findings of the current case together with the relationship between strumal carcinoids and struma ovarii are discussed.     

Uveal metastasis from carcinoid tumor. Clinical observations in nine cases

BACKGROUND: Carcinoid tumor is a low-grade malignancy that usually arises in the gastrointestinal tract or bronchus and rarely metastasizes to the eye. Metastasis of carcinoid tumor to the uvea can be confused clinically with other primary and metastatic uveal tumors. METHODS: The authors reviewed the records of 410 consecutive patients with uveal metastases referred to the Ocular Oncology Service at Wills Eye Hospital to identify those in whom carcinoid tumor was the primary neoplasm. The authors evaluated the clinical features of these metastases. RESULTS: Of 410 consecutive patients with uveal metastases, the primary neoplasm was a carcinoid tumor in 9 (2.2%). There were four men and five women. The mean age at ocular diagnosis was 50 years. In five patients (56%), the primary tumor was undiagnosed at ocular presentation. In the other four patients, the mean time interval from diagnosis of the primary carcinoid tumor to uveal metastasis was 89 months (range, 55-180 months). The site of the primary carcinoid tumor was the bronchus in seven patients, the esophagus in one, and the thymus in one. The site of intraocular metastasis was the choroid in six patients, the ciliary body in two, and the iris in one. All choroidal tumors had a characteristic orange color. Initial ocular treatment included external beam radiotherapy in five patients, plaque radiotherapy in two, argon laser photocoagulation in one, and local resection in one. Ocular tumor control was achieved in each patient. After a mean follow-up of 34 months, four patients (44%) are still alive. Five patients have died, with a mean survival of 34 months (range, 2-104 months) after the diagnosis of uveal metastasis. CONCLUSIONS: Uveal metastasis from carcinoid tumor is rare and tends to arise from the bronchus. Clinically, it has a distinctive orange color and may be associated with a longer systemic survival, compared with uveal metastasis from other primary sites.     

Somatostatin receptor subtypes, octreotide scintigraphy, and clinical response to octreotide treatment in patients with neuroendocrine tumors

Several types of neuroendocrine tumor express high numbers of somatostatin receptors (sstr). We have compared the expression of sstr subtypes with the outcome of octreotide scintigraphy in patients with carcinoids and medullary thyroid carcinoma (MTC) in comparison with Hürthle cell tumors. The effect of sstr activation (octreotide treatment) on tumor markers was also studied in patients with disseminated carcinoid tumors. Six patients with carcinoid tumors (four midgut and two foregut), and three patients with thyroid tumors (one MTC, one Hürthle cell carcinoma, and one Hürthle cell adenoma) were studied. Octreotide scintigraphy visualized tumor sites in all nine patients. Macroscopic tumor was verified at these sites at subsequent surgical exploration. Using Northern blotting and subtype-specific riboprobes, sstr could be detected in all tumors examined. All five sstr subtypes were detected in most of the carcinoid tumors. All six carcinoids expressed sstr2. This was in contrast to the findings for the thyroid tumors analyzed, which also expressed several sstr subtypes but in some cases lacked expression of sstr2. This was also the case for normal thyroid tissue. Clinically, octreotide treatment of the patients with midgut carcinoid tumors resulted in palliation of hormonal symptoms accompanied by a significant reduction of urinary 5-HIAA levels (28-71%). These results indicate that carcinoid tumors frequently express all five sstr subtypes. The thyroid tumors also expressed multiple sstr but could lack expression of sstr2. Nevertheless, these tumors were visualized by octreotide scintigraphy, indicating that sstr2 expression is not a prerequisite for tumor imaging.     

Coexisting undifferentiated thymic carcinoma and thymic carcinoid tumor

Thymic carcinoma is an unusual neoplasm, and the undifferentiated type is rare. Thymic carcinoid is also rare. This report describes a patient with coexisting undifferentiated thymic carcinoma and a carcinoid tumor. Both lesions were completely excised. The carcinoid cells showed argyrophilic granules by Grimelius' method and immunoreactivity against neuron-specific enolase, whereas the undifferentiated carcinoma cells were negative for argyrophilic stain and immunostaining against neuron-specific enolase.