Cases reported "Carcinoma, Medullary"

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1/227. 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. ( info)

2/227. 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. ( info)

3/227. Normal thyroid pathology in patients undergoing thyroidectomy for finding a RETgene germline mutation: a report of three cases and review of the literature.

    Genetic screening for germline RET proto-oncogene mutation in hereditary medullary thyroid cancer (MTC) is accurate and allows for preventive total thyroidectomy to be performed early in patients who are gene carriers. We report 3 children who underwent preventive total thyroidectomy based on the finding of a RETgene germline mutation, but who had no evidence of MTC or C-cell hyperplasia on permanent histology, even after calcitonin immunostaining. review of the English literature of patients undergoing preventive thyroidectomy for a positive RETgene germline mutation, shows that 3.4% of these patients (a total of 209 patients) had normal thyroid glands. Also, 8.6% of patients undergoing preventive total thyroidectomy with prophylactic central neck node dissection had cervical node metastases. We conclude that preventive thyroidectomy in patients screened early for germline RETgene mutation allows for earlier diagnosis and treatment of patients, sometimes even before any hyperplasia or neoplasia can be demonstrated because cervical node metastases can occur early and be demonstrated even with small tumors (< 1 cm), we recommend prophylactic central neck node dissection at the time of preventive thyroidectomy. ( info)

4/227. Unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy in a patient with multiple endocrine neoplasia type 2a syndrome.

    We report the first patient who had bilateral pheochromocytoma associated with multiple endocrine neoplasia type 2a syndrome (men 2a) and underwent unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy 2 years later. The postoperative course was uneventful both times, and the patient was cured of hypertension without any need for steroid replacement. Endoscopic partial adrenalectomy is a minimally invasive procedure for pheochromocytoma with mild symptoms. We believe that this procedure has considerable potential for treating bilateral pheochromocytoma, which is frequently observed in patients with men 2a. ( info)

5/227. 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. ( info)

6/227. Percutaneous dilatational tracheostomy in a patient with thyroid cancer and severe airway obstruction.

    A patient with extensive metastatic thyroid cancer scheduled for palliative tracheostomy is presented. He had laryngeal dislocation with severe airway obstruction and few anatomical landmarks due to tumour infiltration and radiation. Successful percutaneous dilatational tracheostomy was performed under local anaesthesia. ( info)

7/227. 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. ( info)

8/227. 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. ( info)

9/227. 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. ( info)

10/227. 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. ( info)
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