1/23. Aggressive metastatic follicular thyroid carcinoma with anaplastic transformation arising from a long-standing goiter in a patient with Pendred's syndrome.In this article we describe detailed pathological and molecular genetics studies in a consanguineous kindred with Pendred's syndrome. The index patient was a 53-year-old female patient with congenital deafness and goiter. Her parents were first-degree cousins. She had a large goiter (150 g) that had been present since childhood. One of her sisters and a niece are also deaf and have goiter as well. The presence of Pendred's syndrome was confirmed by a positive perchlorate test and the demonstration of a Mondini malformation. thyroid function tests (under levothyroxine [LT4] therapy) were in the euthyroid range with a thyrotropin [TSH] level of 2.8 microU/mL (0.2-3.2), a serum total thyroxine (T4) of 90 nmol/L (54-142), and a serum total triiodothyronine (T3) of 2.7 nmol/L (0.8-2.4). Total thyroidectomy was performed, and the mass in the right lobe was found to have invaded adjacent tissues. The histopathological findings were consistent with a follicular carcinoma with areas of anaplastic transformation and lung metastasis. The patient was treated twice with 100 mCi 131iodine (3,700 MBq) and received suppressive doses of LT4. Postoperatively, the serum thyroglobulin (Tg) levels remained markedly elevated (2,352 to 41,336 ng/mL). The patient died of a sudden severe episode of hemoptysis. sequence analysis of the PDS gene performed with dna from the two relatives with Pendred's syndrome revealed the presence of a deletion of thymidine 279 in exon 3, a point mutation that results in a frameshift and a premature stop codon at codon 96 in the pendrin molecule. We concluded that prolonged TSH stimulation because of iodine deficiency or dyshormonogenesis in combination with mutations of oncogenes and/or tumor suppressor genes, may result in the development of follicular thyroid carcinomas that undergo transformation into anaplastic cancers. It is likely that these pathogenetic mechanisms have been involved in the development of aggressive metastatic thyroid cancer in this unusual patient with Pendred's syndrome.- - - - - - - - - - ranking = 1keywords = carcinoma (Clic here for more details about this article) |
2/23. bloom syndrome in sibs: first reports of hepatocellular carcinoma and wilms tumor with documented anaplasia and nephrogenic rests.The triad of small body size, immunodeficiency, and sun-sensitive facial erythema characterizes the phenotype bloom syndrome (BS), a rare autosomal recessive disorder with a striking predisposition to multiple types of cancers that arise earlier than expected in the general population. Here we report two sibs with BS. The older, a 15-year-old-girl, developed a hepatocellular carcinoma, a neoplasm not yet reported in association with BS. Her younger brother developed an anaplastic wilms tumor (WT) associated with nephrogenic rests at the age of 31/2 years, and this was followed by a myelodysplastic syndrome. Complex cytogenetic abnormalities were identified in all three neoplasms. These examples expand the spectrum of malignancies occurring in BS to include liver cell neoplasms, and confirm the association of nephrogenic rests with WT, even in the setting of BS.- - - - - - - - - - ranking = 0.83333333333333keywords = carcinoma (Clic here for more details about this article) |
3/23. Glandular patterns in a thyroid carcinoma with insular and anaplastic features: a case with possible implications for the classification of thyroid carcinomas.We describe the case of a 33-year-old woman with a thyroid carcinoma showing poorly differentiated (insular), anaplastic, and glandular features, the latter with extensive clear cell changes. Grossly, the well-circumscribed tumor nodule measured 3.6 cm in maximum dimension and was confined to the thyroid. Microscopically, the majority of the tumor was composed of well-defined "insular" nests showing microfollicular formation, high mitotic activity, and areas of necrosis. Other regions, as well as the intervening stroma of the insular nests, were characterized by highly atypical and pleomorphic stromal cells, extensive necrosis, and malignant cartilaginous nodules. Approximately 30% of the tumor was composed of diffuse glandular formations, each of which were lined by elongated, simple columnar cells with basally situated, mildly pleomorphic nuclei, clear supranuclear, periodic acid-Schiff (and diastase sensitive) cytoplasm, empty lumens, and no myoepithelia or basement membranes. Immunohistochemically, the glandular elements displayed diffuse and strong positivity for thyroid transcription factor-1, bcl-2, and CAM 5.2, sparse positivity for thyroglobulin and Ki67, and diffuse but weak positivity for p53. calcitonin was negative throughout the tumor. Karyotypic analysis of a primary culture showed a complex hypertriploid karyotype including structural abnormalities of chromosomes X, 1, 4, 6, 9, 13, and 14 in the majority of cells examined. This composite of histologic findings, especially the glandular patterns, is unusual and their prognostic significance is unclear. The patient is alive with no evidence of tumor recurrence or metastasis at 5 months follow-up. overall, the morphologic and immunohistochemical properties of the glandular component suggests that they are less differentiated than well-differentiated carcinomas and are probably more differentiated than the insular component. This case supports the theory that the various primary carcinomas of the thyroid may represent points along a spectrum rather than distinct entities.- - - - - - - - - - ranking = 1.8333333333333keywords = carcinoma (Clic here for more details about this article) |
4/23. Non-functioning adrenal cortical carcinoma presenting with metastasis to the tongue.We report a case of non-functioning adrenal cortical carcinoma (ACC) presenting with metastatic disease to the tongue, which is an extremely uncommon onset for this neoplasm. Histologically, the lesion had the appearance of an anaplastic neoplasm, and a panel of immunohistochemical markers including vimentin, MART-1, S100 protein, HMB-45, smooth muscle actin, common muscle actin, desmin, CD31, CD34, CD68, EMA and cytokeratins, was helpful in excluding melanoma, as well as other mesenchymal and epithelial neoplasms.- - - - - - - - - - ranking = 0.83333333333333keywords = carcinoma (Clic here for more details about this article) |
5/23. Encapsulated anaplastic thyroid carcinoma without invasive phenotype with favorable prognosis: report of a case.We herein report a case of anaplastic thyroid carcinoma in a 77-year-old woman with long-term disease-free survival. The tumor measured 7.5 x 6.0 cm in size and was diagnosed to be anaplastic carcinoma. We investigated the biological aggressiveness of this carcinoma by means of immunohistochemistry and found it have a high cell-proliferating activity, a disruption in the mechanism of apoptosis, and a high potential of cell spreading, similar to that observed in usual anaplastic carcinomas. The only unique point was that this tumor was encapsulated and no invasion of carcinoma cells beyond the capsule was microscopically observed. To avoid an obstruction of the trachea, a lobectomy without lymph node dissection was performed as a "palliative operation." Although neither adjuvant chemotherapy nor radiotherapy was carried out due to her age, she has nevertheless survived with no evidence of recurrence for 57 months after surgery. The presence of such a type of anaplastic carcinoma should thus be noted by surgeons and pathologists, even though the occurrence of such cases seems to be very rare.- - - - - - - - - - ranking = 1.6666666666667keywords = carcinoma (Clic here for more details about this article) |
6/23. Massive thyroid tumoral embolism from a breast carcinoma presenting as acute thyroiditis.We describe an unusual case of metastatic disease to the thyroid, characterized by massive intra-arterial embolization and clinical presentation as acute thyroiditis. The patient, a 37-year-old woman with a history of breast carcinoma, presented clinically with acute thyroiditis. No nodules were palpable, and fine-needle aspiration cytology of the left lobe was performed. It showed a pleomorphic carcinoma and was followed by total thyroidectomy. histology disclosed a pleomorphic carcinoma diffusely affecting the thyroid gland. It was characterized by an extensive intra-arterial tumoral embolization. immunohistochemistry confirmed the metastatic nature of the neoplasm. This is a very uncommon form of metastatic disease to the thyroid. The ischemia and necrosis associated with the embolic process were most probably responsible for this clinical presentation. This atypical clinical and cytologic presentation may induce confusion with a primary neoplasm, mainly anaplastic thyroid carcinoma.- - - - - - - - - - ranking = 1.3333333333333keywords = carcinoma (Clic here for more details about this article) |
7/23. Multiple metastases to the small bowel from large cell bronchial carcinomas.AIM: Metastases from lung cancer to gastrointestinal tract are not rare at postmortem studies but the development of clinically significant symptoms from the gastrointestinal metastases is very unusual. methods: Formalin-fixed, paraffin-embedded tissues were cut into 5 microm thick sections and routinely stained with hematoxylin and eosin. Some slides were also stained with Alcian-PAS. antibodies used were primary antibodies to pancytokeratin, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, vimentin, smooth muscle actin and CD-117. RESULTS: We observed three patients who presented with multiple metastases from large cell bronchial carcinoma to small intestine. Two of them had abdominal symptoms (sudden onset of abdominal pain, constipation and vomiting) and in one case the tumor was incidentally found during autopsy. Microscopically, all tumors showed a same histological pattern and consisted almost exclusively of strands and sheets of poorly cohesive, polymorphic giant cells with scanty, delicate stromas. Few smaller polygonal anaplastic cells dispersed between polymorphic giant cells, were also observed. immunohistochemistry showed positive staining of the tumor cells with cytokeratin and vimentin. Microscopically and immunohistochemically all metastases had a similar pattern to primary anaplastic carcinoma of the small intestine. CONCLUSION: In patients with small intestine tumors showing anaplastic features, especially with multiple tumors, metastases from large cell bronchial carcinoma should be first excluded, because it seems that they are more common than expected.- - - - - - - - - - ranking = 1.1666666666667keywords = carcinoma (Clic here for more details about this article) |
8/23. Anaplastic transformation from papillary thyroid carcinoma with increased serum CA19-9.A 24-year-old woman presented with anaplastic transformation from papillary thyroid carcinoma with increased serum CA19-9. The patient had been diagnosed as having papillary thyroid carcinoma with lung metastasis at 11 years of age. She received a total thyroidectomy with cervical lymph node dissection followed by iodine-131 ((131)I) therapy over 12 years, but died due to sudden onset of rapid dissemination. Elevated serum CA19-9 was detected in the terminal stage, and anaplastic transformation was confirmed by post-mortem examination. Although there are few clinical reports suggesting a prognostic indicator for anaplastic thyroid carcinoma, CA19-9 may be a useful serum marker for this tumor.- - - - - - - - - - ranking = 1.1666666666667keywords = carcinoma (Clic here for more details about this article) |
9/23. Ovarian mucinous tumor with mural nodules of anaplastic carcinoma.A case of mucinous cystic ovarian tumor with mural nodules of anaplastic carcinoma in a 30-year-old woman is described. The carcinomatous components within the nodules showed strong immunopositivity for cytokeratin and carcinoembryonic antigen, and ultrastructurally they displayed epithelial and glandular differentiation. Omental metastasis had already developed in the patient, and she received postoperative adjuvant chemotherapy consisting of cyclophosphamide and cis-platinum. No sign of recurrence was evident 4 months after the operation. The literature is reviewed and the importance of adjuvant chemotherapy in the postoperative management of such patients highlighted. The salient pathologic features differentiating mural nodules of anaplastic carcinoma and true sarcoma from prognostically favorable sarcoma-like nodules are presented.- - - - - - - - - - ranking = 1.1666666666667keywords = carcinoma (Clic here for more details about this article) |
10/23. Small cell carcinoma of prostate.Small cell anaplastic carcinoma (SCC) involving the prostate is extremely unusual, either as a primary or as a secondary metastatic focus. A case of SCC presenting as prostatic obstruction and diagnosed with prostate biopsy is presented and the literature reviewed.- - - - - - - - - - ranking = 0.83333333333333keywords = carcinoma (Clic here for more details about this article) |
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