1/205. Prolonged survival of a patient with sickle cell trait and metastatic renal medullary carcinoma.PURPOSE: The treatment and outcome of a patient with sickle cell trait and metastatic renal medullary carcinoma is described. PATIENT AND methods: A 12-year-old boy with sickle cell trait had metastatic renal medullary carcinoma. After surgical resection of the primary tumor, he received chemotherapy with methotrexate, vinblastine, doxorubicin, and cisplatin. The carcinoma progressed after a 6-month period of stable disease. At that time, he received chemotherapy including ifosfamide, etoposide, carboplatin, and topotecan. RESULTS: The patient died of progressive disease 15 months from diagnosis. The patient's tumor in this report showed no progression while he was receiving methotrexate, vinblastine, doxorubicin, and cisplatin, but eventually became refractory to these and other cytotoxic agents. CONCLUSION: Renal medullary carcinoma is a highly chemotherapy-resistant tumor. Average survival after diagnosis is 15 weeks; the longest survival reported in the literature is 12 months from diagnosis. The patient in this report survived longer than the previously described patients before dying from progressive disease.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/205. Medullary thyroid carcinoma with multiple hepatic metastases: treatment with transcatheter arterial embolization and percutaneous ethanol injection.A 54-year-old man with medullary thyroid carcinoma in the thyroid gland was unable to undergo total thyroidectomy because the tumor had invaded the mediastinum. radiation therapy and chemotherapy were given. Seven years later, intractable diarrhea and abdominal pain appeared, and computed tomography demonstrated hypervascular tumors in the thyroid gland and in the liver. The tumors were successfully treated with percutaneous ethanol injection to a lesion in the thyroid gland and transcatheter arterial embolization followed by percutaneous ethanol injection to tumors in the liver. Transcatheter arterial embolization and percutaneous ethanol injection may be valuable in treating medullary thyroid carcinoma.- - - - - - - - - - ranking = 0.75keywords = carcinoma (Clic here for more details about this article) |
3/205. Gastroenteropancreatic neuroendocrine tumor metastases to the thyroid gland: differential diagnosis with medullary thyroid carcinoma.neuroendocrine tumors (NET) of the thyroid gland are rare. Apart from medullary thyroid carcinoma (MTC), metastases of gastroenteropancreatic (GEP) NET may also occur. Features of six patients (five men, one female: age range, 39-67 years) with thyroid metastases from a GEP-NET are described. Thyroid metastases were bilateral in all patients and were associated with enlarged neck lymph nodes in five. In four cases, the thyroid tumor was either the first sign of the disease (n = 2) or was an isolated site of recurrence (n = 2). The tumors were well (n = 3) or poorly differentiated (n = 3). Five tumors for which the primary site could be determined corresponded to foregut-derived tumors (3 lungs, 1 thymus and 1 pancreatic NET). One tumor demonstrated calcitonin (CT) production as shown by immunohistochemistry and elevated plasma CT levels. However, the disease history and the clinical features strongly favored a metastasizing GEP-NET. No tumoral RET proto-oncogene mutation was found in this patient. The differential diagnosis between metastatic GEP-NET and MTC is crucial because prognosis, work-up, and treatment differ greatly.- - - - - - - - - - ranking = 0.625keywords = carcinoma (Clic here for more details about this article) |
4/205. multiple endocrine neoplasia type 2a with the identical somatic mutation in medullary thyroid carcinoma and pheochromocytoma without germline mutation at the corresponding site in the RET proto-oncogene.A germline mutation either in exon 10 or 11 of the RET proto-oncogene is found in the majority of patients with multiple endocrine neoplasia type 2a (men 2A). A 41-year-old female patient was referred for further evaluation of incidentally discovered right adrenal tumor. She had bilateral adrenal pheochromocytomas and medullary thyroid carcinomas detected by endocrinological and radiological examination, and diagnosed as men 2A. Molecular genetic testing of the RET exons 10 and 11 exhibited the identical somatic missense mutation at codon 634 in both tumors but did not confirm germline mutations in the corresponding sites. Possible mechanisms for tumorigenesis in this patient are discussed.- - - - - - - - - - ranking = 0.625keywords = carcinoma (Clic here for more details about this article) |
5/205. Hepatic metastases from medullary thyroid carcinoma appearing twelve years after the eradication of primitive tumor: cytological and radiological aspects.We report on radiological and cytological findings from a case of medullary thyroid carcinoma (MTC) metastatizing to the liver 12 yr after the eradication of the primary neoplasm. This behavior has never before been described in a sporadic form of MTC.- - - - - - - - - - ranking = 0.625keywords = carcinoma (Clic here for more details about this article) |
6/205. Thyroid C-cell carcinoma with amyloid in a red fox (Vulpes vulpes schrenchki).An amyloid-producing medullary thyroid carcinoma (MTC) in a red fox (Vulpes vulpes schrenchki) bred in a zoo was examined using histopathologic and immunohistochemical techniques. The neoplastic cells had an ill-defined cytoplasmic membrane and abundant, finely granular eosinophilic cytoplasm, containing numerous argyrophilic granules. The neoplastic tissues were divided into various sizes by a vascular connective stroma, which was partly fibrovascular with broad areas of hyalinization containing varied amounts of amyloid. Immunohistochemically, neoplastic cells showed reactivity to anti-calcitonin, neuron-specific enolase, somatostatin, and keratin antibodies. However, amyloid in the stroma did not show immunoreactivity to the antibodies used. Histologic and immunohistochemical features of MTC in the present animal were analogous to those of the C-cell carcinoma derived from thyroid C cells (parafollicular cells) reported in humans and dogs.- - - - - - - - - - ranking = 0.75keywords = carcinoma (Clic here for more details about this article) |
7/205. Occult sporadic medullary microcarcinoma with lymph node metastases.A sporadic form of medullary occult microcarcinoma was cytologically diagnosed, by fine-needle aspiration biopsy (FNAB), on metastatic cervical lymph nodes. The cytologic specimens were partially smeared and partially zinc-formalin-fixed, so that a microinclusion clot for immunohistochemical studies would be possible. The reactivity to calcitonin of neoplastic cells obtained from metastatic cervical lymph nodes supported the search for a thyroid tumor, which only a careful echographic study revealed. Under sonographic guidance, FNAB on a 0.5-cm hypoechoid nodule was performed. The smears were diagnostic of medullary carcinoma. A total thyroidectomy with node dissection was made. Diagn. Cytopathol. 1999;21:203-206.- - - - - - - - - - ranking = 0.75keywords = carcinoma (Clic here for more details about this article) |
8/205. somatostatin receptor scintigraphy for early detection of regional and distant metastases of medullary carcinoma of the thyroid.Three patients are described who had regional and distant metastases of medullary thyroid cancer detected by somatostatin receptor scintigraphy but not by CT; two had minimal disease that was amenable to surgery. The first patient had been followed for 2 years before having a repeated scan and positive CT, with subsequent surgical removal of metastatic paratracheal nodes. The plasma calcitonin level, however, did not approach normal values after surgery, and a third scan showed persistence of focal uptake in the left paratracheal area of the lower neck, whereas CT was negative. At repeated exploration, a tumor mass of medullary carcinoma, embedded in lymphatic tissue, was removed. Nine months after the last surgical procedure, calcitonin and carcinoembryonic antigen levels were normal. The second patient underwent microdissection of the mediastinum and removal of two metastatic nodes that were demonstrable only by the scintigraphic technique. The plasma calcitonin level subsequently became normal. The third patient, with multiple endocrine neoplasia IIB and associated pheochromocytoma, had bony metastatic involvement of the left shoulder, demonstrable initially on somatostatin receptor scintigraphy and subsequently with radioiodinated metaiodobenzylguanidine but not on CT.- - - - - - - - - - ranking = 0.625keywords = carcinoma (Clic here for more details about this article) |
9/205. Incidental detection of familial medullary thyroid carcinoma by calcitonin screening for nodular thyroid disease.serum calcitonin screening has recently been found to be a useful supplement to fine-needle aspiration biopsy, ultrasound and radionuclide imaging in the evaluation of thyroid nodules. We describe a case where introduction of routine calcitonin screening in nodular thyroid disease led to the detection of a family with medullary thyroid carcinoma. The benefits and problems of basal and stimulated serum calcitonin testing and ret-proto-oncogene mutation studies are exemplified and we discuss the appropriate use and interpretation of these tests. We conclude that routine basal serum calcitonin measurement in nodular thyroid disease and thoughtful use of ret-mutation analysis is cost-effective in detecting medullary thyroid carcinoma and multiple endocrine neoplasia type II.- - - - - - - - - - ranking = 0.75keywords = carcinoma (Clic here for more details about this article) |
10/205. Familial medullary thyroid carcinoma: presymptomatic diagnosis and management in children.Two kindreds with familial medullary thyroid carcinoma (MTC) are described in which affected family members had variable clinical and pathologic manifestations. genetic testing in 2 children from one kindred revealed a mutation in exon 10, codon 618 (TGC to AGC) in the extracellular cysteine-rich region of the RET gene. In this kindred an 11-year-old had microscopic evidence of MTC; however, a 17-year-old had no evidence of pathology on thyroidectomy. In a second kindred a rare mutation in exon 14, codon 804 (GTG to TTG) of the intracellular tyrosine kinase region of the RET gene was detected. In this kindred MTC has occurred in the 4th to 5th decades of life, with a clinical spectrum in mutation-positive family members ranging from no disease and C-cell hyperplasia to carcinoma with lymph node metastasis; a 7-year-old with the mutation and a normal response to provocative testing was also identified. Management recommendations in children from families with clearly defined familial MTC may be individualized to reflect emerging genotype-phenotype correlations.- - - - - - - - - - ranking = 0.75keywords = carcinoma (Clic here for more details about this article) |
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