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.     

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.     

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.     

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 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.     

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.     

Liver metastasis from carcinoid: diagnostic imaging

Our study was aimed at describing the diagnostic imaging patterns of carcinoid liver metastases. Six patients with liver metastases secondary to carcinoid tumor were examined. The metastases were histologically proved in each patient. All patients were examined with ultrasonography (US), Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and Digital Subtraction Angiography (DSA). All patients were treated with transcatheter embolization of liver metastases. Diagnostic imaging methods showed ten to many dozen metastases in each patient. Tumor size ranged 0.5 to 14 cm. US showed hypoechoic, hyperechoic and isoechoic carcinoid liver metastases with a hypoechoic halo. Large lesions had anechoic central areas due to colliquative necrosis. Hypoechoic patterns were the most frequent ones. Precontrast CT showed hypodense metastases; very small lesions were isodense relative to surrounding liver. CT during contrast agent injection showed that the metastases were hyperdense in the arterial phase; contrast enhancement was poorer in the portal phase. Large lesions showed a hypodense central area due to necrosis which remained hypodense in the late phase. The metastases were hypointense on T1-weighted and hyperintense on T2-weighted MR images. Gradient echo dynamic imaging with Gd-DTPA showed high-signal metastases in the arterial phase and lower signal intensity in the portal phase. DSA, an essential exam before embolization, showed tortuous and elongated intra- and extrahepatic arteries and tumor neovascularization with no malignant abnormalities. In the capillary phase, tumor uptake and inhomogeneous hypervascular patterns were shown. Portal veins were only displaced and compressed, but never infiltrated by the metastases. All the techniques we used contributed to assess liver involvement by carcinoid metastases. DSA must be used only before treatment; both CT and MRI showed the hypervascular patterns of the metastases, but no technique could predict their nature.     

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.     

Solitary breast metastasis as first manifestation of ovarian carcinoid tumor

Carcinoid tumor metastatic to the breast is very uncommon. The rarity of this finding may be the reason for its common misinterpretation as primary breast carcinoma. A patient with primary ovarian carcinoid that presented initially with a solitary breast mass is reported. A review of the literature discloses only 12 reported cases of carcinoid tumor metastatic to the breast, with none of the primaries found to be the ovary.     

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.     

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.     

Subacute orbital abscess in a four-year-old girl with a new kitten

BACKGROUND: Carcinoid tumors are neoplasms of neuroendocrine origin that rarely affect the genital tract. CASE: A 75-year-old woman underwent hysterectomy and bilateral adnexectomy due to vaginal bleeding and uterine pathology (leiomyoma, cervical low grade squamous intraepithelial lesions and endometrial hyperplasia on ultrasound). Pathologic examination of the specimen disclosed a uterine corpus carcinoid tumor. The patient had been taking tamoxifen for adjuvant treatment of breast cancer diagnosed and treated seven years before. CONCLUSION: A review of the literature revealed one case of carcinoid tumor of the uterine wall. There does not appear to be any relationship between tamoxifen and the carcinoid tumors reported.     

Imported fever

OBJECTIVE: The curative potential of various bronchoscopic treatments such as Nd:YAG laser, photodynamic therapy, and brachytherapy for the treatment of intraluminal tumor has been reported previously. Bronchoscopic treatment can be used to treat small intraluminal tumor with curative intent, such as in patients with roentgenologically occult squamous cell cancer. In a retrospective study, we showed that bronchoscopic treatment provided excellent local control with surgical proof of cure in 6 of 11 patients with intraluminal typical bronchial carcinoid. METHODS: In a prospective study, 19 patients (8 women and 11 men) with resectable intraluminal typical bronchial carcinoid have undergone bronchoscopic treatment under general anesthesia. Median age was 44 years (range, 20-74 years). If tumor persisted after 2 bronchoscopic treatment sessions, surgery was performed within 4 months after the treatment. RESULTS: Bronchoscopic treatment was able to completely eradicate tumor in 14 of the 19 patients (complete response rate 73%, 95% CI: 49%-91%). Median follow-up of these patients is 29 months (range, 8-62 months). One patient had severe cicatricial stenosis after bronchoscopic treatment, and sleeve lobectomy was necessary. No residual carcinoid was found in the resected specimen. In the remaining 5 patients, bronchoscopic treatment did not result in a complete response and radical surgical resection was performed afterward with confirmation of residual carcinoid in the resected specimen. Median follow-up of the surgical group is 34 months (range, 12-62 months). CONCLUSIONS: Current data suggest that bronchoscopic treatment may be an effective alternative to surgical resection in a subgroup of patients with resectable intraluminal typical bronchial carcinoid. It alleviated the necessity of surgical resection in 68% (95% CI: 43%-87%) of the patients.     

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.     

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.     

Multimodality treatment for gastric carcinoid tumor with liver metastases

Carcinoid tumors are the most common neuroendocrine tumors in the gastrointestinal tract, and between 10% and 30% of these tumors are gastric in origin. Three types of gastric carcinoid tumors are recognized: type I, associated with chronic atrophic gastritis type A; type II, associated with multiple endocrine neoplasia; and type III, sporadic and the most malignant. We present a patient with an aggressive, sporadic-type gastric carcinoid that metastasized to the liver. Her symptomatic treatment included the somatostatin analog octreotide. Octreotide scintigraphy demonstrated that this tumor avidly bound the peptide. The patient's gastric carcinoid (assessed by endoscopy and endoscopic ultrasound) regressed and she underwent hepatic artery embolization for her liver metastases. After initial partial CT resolution the tumor grew, compressing the inferior vena cava. The patient underwent orthotopic liver transplant with excellent recovery, although she was subsequently found to have two small lung metastases. She has responded well to adjuvant Indium-111 octreotide receptor targeted therapy. This case highlights the therapeutic options for metastatic neuroendocrine tumors, including liver transplantation and adjuvant receptor targeted therapy.     

Anatomical variants during axillary dissection

PURPOSE: To report a minimally symptomatic patient with bilateral carcinoid tumors metastatic to the choroid who was followed up for 42 months without any treatment. METHOD: Case report. RESULTS: A 54-year-old white man with a history of bronchial carcinoid tumor had bilateral multiple carcinoid tumors metastatic to the choroid. Follow-up for 42 months without treatment demonstrated no growth of the choroidal tumors, and corrected visual acuity remained RE, 20/20 and LE, 20/25. Systemic status also remained stable. CONCLUSIONS: Carcinoid tumors metastatic to the choroid may remain stationary for an extended period. Treatment may be deferred until tumor growth, exudative retinal detachment, or substantial impairment of vision develops.     

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.     

Goblet cell carcinoid of the appendix. An ultrastructural and histochemical study

In a patient with a goblet cell carcinoid tumor of the appendix, light and electron microscopical studies demonstrated mucinous material and enterochromaffin granules within the same cell. Transitions between cells containing primarily mucin and cells containing numerous argentaffin granules were observed. Fluorescence studies demonstrated that the tumor cells contain biogenic amines.     

Carcinoid tumor of the gallbladder

The carcinoid tumor is a relatively rare type of endocrine tumor, which arises mainly in the gastrointestinal tract. Primary gallbladder and biliary duct system carcinoids comprise less than 1% of all carcinoid tumors arising from any tissue or organ in the body. We describe a case of carcinoid tumor of the gallbladder in a 39-year-old man. There have been only 32 cases described in the literature.