Pseudocystic metastases of carcinoma of the uterine cervix mimicking polycystic liver disease

Pseudocystic liver metastases are rare and mainly described in neuroendocrine or ovarian tumors. We report the case of a 46-year-old woman who presented with multiple hepatic metastases mimicking polycystic liver disease. Carcinoma of the uterine cervix had been diagnosed 9 years earlier, and initially treated by radiumtherapy and surgery. Although histological post mortem examination of the pseudocystic liver metastases was not characteristic, they were related to the uterine cervix carcinoma for the following reasons: no other primary tumor was discovered, especially carcinoid or ovarian tumors: immunostains were positive for epithelial cells and negative for the neuroendocrine panel: the cystic cerebellum metastasis had a typical histologic aspect. Uterine cervical carcinoma must thus be included in the list of tumors which may form cystic hepatic metastases.     

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.     

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.     

Primary myxoid leiomyoma of the liver

We describe a case of hepatic leiomyoma in a 41-year-old woman who complained of abdominal discomfort due to a right upper quadrant abdominal mass. A computed tomographic scan revealed a large hepatic mass with unusual cystic change. Light and electron microscopic findings and an immunohistochemical study demonstrated diffuse and scattered proliferation of smooth muscle cells in a myxoid matrix. In contrast to previously reported cases of primary leiomyoma of the liver, the present tumor developed in a patient without an immunosuppressive condition. To the best of our knowledge, this is the first reported case of primary "myxoid" leiomyoma of the liver.     

Hemangiosarcoma of liver and spleen treated by hepatic artery ligation, intraportal infusion chemotherapy, and splenectomy

Hepatic artery ligation is useful as a palliation of irressectable hepatic tumors, but does not always produce a satisfactory result. Hepatic tumor with high vascularity is expected to respond more favorably. In this context, primary liver cell carcinoma and carcinoid tumor or leiomyosarcoma of the liver have been satistfactorily treated by hepatic artery ligation. A case is presented of hemangiosarcoma of theliver and spleen treated effectively by hepatic artery ligation, splenectomy, and postoperative intraportal infusion of 5-fluorouracil, as indicated by the regression of hepatic tumors on postoperative scanning and arteriograpms.     

Expression of prohormone convertase, PC2, in adrenocorticotropin-producing thymic carcinoid with elevated plasma corticotropin-releasing hormone

An autopsy case of ACTH-producing thymic carcinoid with Cushing's syndrome is reported. The patient was a 63-yr-old man with multiple bone metastases from an undetermined primary site. Hyperpigmentation was observed at the terminal stage. The plasma levels of ACTH, cortisol, chromogranin A, and urinary 17-hydroxy-corticosteroids were extremely high, and ectopic ACTH-producing neuroendocrine tumor was diagnosed. In addition, plasma CRH was high. Autopsy revealed that the patient had primary thymic carcinoid with extensive metastases. Remarkable hyperplasia of the adrenal cortexes and Crooke's hyaline degeneration of the pituitary gland were consistent with Cushing's syndrome by ectopic ACTH production. There were multiple CRH-producing cells without degenerative changes in the hypothalamus. The tumor cells were immunoreactive to ACTH, CRH, and the cleavage enzyme PC2. POMC messenger ribonucleic acid and PC2 messenger ribonucleic acid were detected in the tumor cells by an in situ hybridization method. Expression of PC2 was considered to induce hyperpigmentation by producing alpha MSH. Despite hypercortisolism and ectopic production of CRH by the tumor cells, hypothalamic CRH cells were not atrophic. This case is a good example to demonstrate the correlation between CRH and the hypothalamo-pituitary-adrenal axis as well as hyperpigmentation in Cushing's syndrome.     

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.     

Inhibitors of mitochondrial energy production prevent DNA internucleosomal fragmentation in thymocytes

We describe a case of multiple hepatic pyogenic abscesses with an unusual presentation. The typical signs and symptoms of fever and pain in the right upper quadrant were absent. Instead, the chief complaint was muscle weakness and myalgias accompanied by weight loss. Findings from an ultrasonogram of the abdomen revealed multiple hepatic lesions consistent with metastases. Hence, the initial presumptive diagnosis was metastatic malignancy with unknown primary tumor. It was only when purulent material was unexpectedly encountered when a needle biopsy was performed that the true diagnosis of pyogenic liver abscess was recognized. While liver abscess is rare, it should not be forgotten in the differential diagnosis for multiple hepatic lesions seen on imaging studies.     

Hemobilia caused by a giant benign hemangioma of the liver: report of a case

We report herein the extremely unusual case of a 39-year-old woman in whom a giant cavernous hemangioma caused hemobilia. Cavernous hemangioma is the most common benign neoplasm of the liver and rarely causes any clinical symptoms or signs, while hemobilia usually occurs secondary to accidental operative or iatrogenic trauma, vascular disease, inflammatory disorders, gallstones, or tumors of the liver. Although invasive or malignant hepatic tumors often result in a communication between the biliary tract and the blood vessels, only one case of hemobilia caused by a benign cavernous hemangioma has ever been reported, but with no details about the patient. Our patient presented to a local hospital with severe melena as the initial main symptom, where ligation of the right hepatic artery was performed. This failed to relieve her symptoms, and she was subsequently referred to our department where a right hepatectomy was performed. Histopathological examination revealed no malignancy combined with the tumor; however, the hemangioma was exposed to the bile duct in segment VIII, which was presumably the cause of the hemobilia. This patient remains in good health almost 6 years after her operation. To the best of our knowledge this is the first case report of hemobilia caused by a cavernous hemangioma, and is accompanied by a detailed analysis.     

Topical carbonic anhydrase inhibition increases ocular pulse amplitude in high tension primary open angle glaucoma

We report herein an unusual case of a composite glandular-neuroendocrine carcinoma of the hilar bile duct. A 71-year-old Japanese woman was admitted to our hospital suffering from general fatigue, progressive jaundice, and a high fever. Computed tomography and angiography findings revealed a solid hypervascular mass in the hepatic hilus. Thus, a subsegmentectomy of the liver (S4, S5) and bile duct resection with lymph node dissection were performed. A tumor measuring 6.0 x 3.0 cm was found to be located in the bile duct of the hepatic hilus. Histologically, the tumor was composed of well-differentiated adenocarcinoma and small cell neuroendocrine carcinoma cells, with a histological transition between the two components. Grimelius' method revealed the presence of diffuse positive tumor cells in neuroendocrine carcinoma. The neuroendocrine tumor cells were also diffusely immunoreactive to chromogranin A. To the best of our knowledge, only 22 previous cases of composite glandular-neuroendocrine carcinoma in the biliary tract have been reported; however, this is the first case report of a clearly composite tumor of the hilar bile duct.     

Genotator: a workbench for sequence annotation

The clinical, pathological, and immunohistochemical features of six cases of metastatic neuroendocrine and carcinoid tumors to the thyroid simulating medullary thyroid carcinoma (MTC) are described. The patients were women between the ages of 24 and 70 years who, without symptoms or significant past medical histories, presented with either a single mass or multiple thyroid nodules. The primary source of the tumor was only discovered on follow-up. Two of the neoplasms were classical carcinoid tumors, one was a carcinoid predominantly composed of large cells, another showed a prominent oval to spindle cell component, and the two remaining cases were atypical carcinoid/high-grade neuroendocrine carcinomas. The immunohistochemical profile was inconsistent with MTC in that all tumors were negative for calcitonin and only two were focally positive for carcinoembryonic antigen (CEA). A variable pattern of staining for other neuroendocrine and epithelial markers was obtained in each case. Despite the morphologic and immunohistochemical similarities with MTC, the diagnosis of a metastatic neuroendocrine tumor to the thyroid should be favored in the presence of a predominantly interstitial pattern of spread; occurrence of multiple tumor foci; folliculotropism; rosette formations with lumen and cuticular borders; and lack of immunoreactivity for calcitonin and CEA. The differential diagnosis between MTC and metastatic neuroendocrine carcinoma to the thyroid is of importance because of the vast differences in treatment and prognosis.     

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.     

Acute liver necrosis induced by iodine-131-MIBG in the treatment of metastatic carcinoid tumors

Iodine-131-metaiodobenzylguanidine (MIBG) is used in the treatment of carcinoid tumors. Temporary palliation with complete subjective symptomatic response has been reported in these patients. This treatment is usually well tolerated and side-effects are generally limited to nausea, mild hepatic toxicity with spontaneous recovery and temporary myelosuppression. Our case report shows that repeated treatment with [131I]MIBG in a patient with extensive carcinoid liver metastasis may cause severe hepatic toxicity leading to death. Factors such as concomitant use of 5-fluorouracil and the progressive nature of the disease may have contributed to this event.     

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.     

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.     

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.     

Fibrolamellar carcinoma of the liver with a mixture of ordinary hepatocellular carcinoma: a case report

Fibrolamellar carcinoma (FLC) of the liver is a rare variant of hepatocellular carcinoma (HCC), and only 13 cases have been reported in Japan up to 1997. We described a histologically unusual case of FLC. A 52-yr-old man was admitted to our hospital for work-up of hepatic mass. Laboratory examinations revealed no abnormalities except elevated serum alpha-fetoprotein (AFP) (2098 ng/ml). A diagnosis of HCC was made by imaging findings, and left lobectomy of the liver was performed. Histologically, the tumor was composed of areas of FLC mixed with ordinary HCC and those of pure ordinary HCC. Staining for AFP was positive in the HCC component and negative in the FLC component. Some cases of such mixed tumors have been reported in Europe and the United States, but not in Japan. We regarded our case as the first of the mixed tumor in Japan.     

Concurrent hypercortisolism and hypermineralocorticoidism

The clinical and pathologic findings in 16 cases of carcinoid tumor of the testis are presented: 10 tumors were primary in the testis, 2 were teratomas, 2 were in the spermatic cord and 2 were metastatic. Most of these tumors occurred in middle-aged patients and the symptoms were those of testicular tumor in general, that is swelling, pain and tenderness. In none of the primary case was there evidence of carcinoid syndrome and no determination of serotonin was made before orchiectomy. Followup in 12 cases was from 4 months to 16 years postoperatively. Three patients died 2 to 4 years after orchiectomy: 1 with generalized metastasis and 2 with intercurrent diseases. Two patients were lost to followup and the remaining patients are well. Although ovarian carcinoids usually occur in teratoma and do not metastasize 1 of the primary cases produced generalized metastasis.