1/100. Multiple endocrine neoplastic-associated thymic carcinoid tumour in close relatives: octreotide scan as a new diagnostic and follow-up modality. Two case reports.Thymic carcinoid tumours constitute less than 1% of all carcinoids, and differ markedly from true thymomas in natural history, morphology, prognosis and therapeutic options. New clinical and diagnostic modalities are described in two brothers with thymic carcinoid associated with multiple endocrine neoplasia syndrome. octreotide scintigraphy proved useful for diagnosis and follow-up, and somatostatin receptor positivity may provide new prospects for treatment of non-resectable or recurrent tumour.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/100. 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 = 1keywords = endocrine (Clic here for more details about this article) |
3/100. Angiomatoid neuroendocrine carcinoma of the thymus: report of a distinctive morphological variant of neuroendocrine tumor of the thymus resembling a vascular neoplasm.Three cases of primary thymic neuroendocrine tumors characterized by prominent angiomatoid features that resembled a vascular neoplasm are presented. The patients were all men between 52 and 59 years of age who presented with chest pain and shortness of breath attributable to a large anterior mediastinal mass. The lesions ranged in size from 6 cm to 15 cm in greatest diameter, and were grossly soft and well circumscribed, but not encapsulated. The cut surface was remarkable for multiple blood-filled cyst-like spaces admixed with focal solid, hemorrhagic areas. Histologically, the tumors contained multiple cystically dilated spaces filled with blood which imparted the lesion with a striking angiomatoid appearance. The walls of the cysts were lined by a monotonous proliferation of round to oval cells with distinct cell borders, round central nuclei, and abundant eosinophilic cytoplasm. Mitotic activity was present in all cases and varied from 3 to 8 mitoses per 10 high-power fields. Immunohistochemical studies performed in two cases showed positivity of the tumor cells for keratin, Leu 7, and synaptophysin, and focal chromogranin positivity in one. Follow-up information obtained in two patients showed that both had died of tumor 4 and 8 years after initial diagnosis. The present cases show an unusual morphological appearance of thymic neuroendocrine tumors that may be mistaken for a vascular neoplasm. Immunohistochemical stains may be of importance in such instances in arriving at the correct diagnosis.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
4/100. A family of MEN1 with a novel germline missense mutation and benign polymorphisms.The gene responsible for multiple endocrine neoplasia type 1 (MEN1) has recently been cloned, and its germline mutations were identified in patients with this syndrome. The majority of the mutations, frameshift or nonsense mutations, are expected to result in a loss of function of the gene product menin. Since the consequence of less common missense or in-frame deletion mutations is not clear, careful judgment is necessary regarding the role(s) of such mutations in MEN1 disease. Here we describe a large multigenerational MEN1 family with a novel germline missense mutation and three benign polymorphisms. The proband was a man with hyperparathyroidism and thymic carcinoid. We performed biochemical studies and dna analyses of the MEN1 gene simultaneously and independently as family screening studies. Seven patients including the proband were identified, and all of them carried a heterozygous germline missense mutation E45G, but 5 members with normal biochemical results did not. This mutation was not observed in 50 normal volunteers. This novel missense mutation is therefore almost conclusively responsible for the disease. Although all of the mutant gene carriers in the present study already had clinical diseases, an MEN1 gene analysis in younger individuals at risk would be very useful in identifying carriers before the onset of the symptoms.- - - - - - - - - - ranking = 0.2keywords = endocrine (Clic here for more details about this article) |
5/100. Granulocytic sarcoma of the thymus in a nonleukaemic patient.We report a case of granulocytic sarcoma arising from the thymus in a 17-year-old nonleukaemic patient. The patient presented with an anterior mediastinal tumour and underwent surgical resection. Histological examination showed a diffuse infiltrate of immature round cells in the thymus. Tumour cells were diffusely peroxidase positive, but naphthol AS-D chloroacetate esterase negative. Immunohistochemical staining revealed expression of CD34 and terminal deoxynucleotidyl transferase (TdT), but not of CD13 and CD33. Ultrastructurally, electron-dense or medium-density granules were present in the cytoplasm. Four months after successful autogenic bone marrow transplantation, pleural and pericardial fluid contained tumour cells with azurophilic granules, which expressed CD13 and CD33, but not CD34 and TdT. The patient died of the disease 18 months after clinical manifestation, but still without developing leukaemia. The granulocytic sarcoma in the present case may have originated from myeloid precursors in the thymus and remained within the extramedullary site despite the differentiation into a more committed myeloid lineage at the relapse.- - - - - - - - - - ranking = 0.001709552596517keywords = bone (Clic here for more details about this article) |
6/100. Macrocytic anemia and thrombocytosis associated with thymoma: a case report.Thymomas are often associated with autoimmune disorders. We report on a 45-year-old female patient with thymoma and hypogammaglobulinemia (Good's syndrome) who developed symptomatic macrocytic anemia (Hb 4.4 g/dl, MCV 112 fl) and thrombocytosis (Plt 442 G/l). Besides hypogammaglobulinemia (IgG 589 mg/dl), an inverted ratio of CD4( )/CD8( ) cells was seen. The bone marrow biopsy showed a slightly hypercellular bone marrow with normal granulopoiesis, normal megakaryopoiesis and a mild dyserythropoiesis without any ring-sideroblasts. The in-vitro stem cell culture from the bone marrow revealed an atypical growth of macroclusters, reduced BFU-E and CFU-GEMM colony growth, whereas the CFU-GM colony growth was within the normal range. The chromosomal analysis showed a normal karyotype. The plasma vitamin B(12) and folate levels were within normal ranges, and we could not detect any autoantibodies. These findings excluded the differential diagnoses pure red cell aplasia (PRCA) and pernicious anemia. After resection of the thymoma of mixed cell type, the macrocytic anemia and thrombocytosis disappeared. The clinical course was complicated by a cerebral palsy and a life-threatening fungal septicemia after surgery. In the third year after thymectomy, hyporegenerative macrocytic anemia and thrombocytosis reappeared and an immunosuppressive treatment with prednisolone (1 mg/kg BW) was started. After initiation of the prednisolone therapy, reticulocyte counts increased and macrocytic anemia as well as thrombocytosis disappeared. The normalization of these laboratory parameters during glucocorticoid therapy suggests that in rare cases the constellation of macrocytic anemia, thrombocytosis and hypogammaglobulinemia may be due to an underlying immunologic mechanism.- - - - - - - - - - ranking = 0.0051286577895511keywords = bone (Clic here for more details about this article) |
7/100. Thymic carcinoid and parathyroid hyperplasia detection with 99mTc-MIBI men type 1.We report a case of a 35-year-old male, with a history of diarrhea, renal lithiasis with frequent expulsions of calculus and hypercalcemia during the last 2 years. The patient was studied and diagnosed with a multiple endocrine neoplasia type I (MEN I), familiar (mother with MEN I). A scintigraphic study with 99mTc-MIBI was performed in order to localize hyperfunctioning parathyroid glands because of biochemical diagnosis of primary hyperparathyroidism. Double phase 99mTc-MIBI scan detected one hyperfunctioning parathyroid gland and a large anterior mediastinal mass. Subsequent, plain radiograph and CT of the chest showed a soft-tissue mass in that localization. Punch biopsy of the lesion guided by CT revealed malignant cells of neuroendocrine tumor. The tumor was removed and histologically confirmed as a carcinoid within a thymus in a MEN type I syndrome. MEN I patients can benefit from the examination with this agent which can potentially localize not only parathyroid endocrine pathology but also unknown associated tumors.- - - - - - - - - - ranking = 0.6keywords = endocrine (Clic here for more details about this article) |
8/100. Malignant thymoma with metastases to the gastrointestinal tract and ovary: a case report and literature review.This is the first clinical case report of a thoracic invasive thymoma metastatic to the ovary with disease noted in the entire abdomen including the pelvis 5 1/2 years after initial diagnosis. The involved areas of metastases include the distal ileum, peritoneal and serosal surfaces (including the surface of the distal colon, bladder, and pelvis), and the surface of the right ovary. The patient survived 13 years after her initial diagnosis and 7 1/2 years after discovery of her metastases. Thymomas are rare tumors but comprise the most common primary tumor of the anterior mediastinum. Extrathoracic metastases of malignant thymomas are also rare, and the literature reports that the most common sites for metastases are the liver, lung, lymph nodes, and bone. Extrathoracic disease is associated with a poor prognosis. The average time of survival after the diagnosis of metastases is 1.5 years.- - - - - - - - - - ranking = 0.001709552596517keywords = bone (Clic here for more details about this article) |
9/100. Uptake of 99mTc-tetrofosmin, 99mTc-MIBI and 201Tl in malignant thymoma.99mTc-tetrofosmin, thallium-201-chloride (201Tl) and 99mTc-MIBI imagings were performed in a patient with malignant thymoma. Tracer uptake in the primary tumor was demonstrated. The tumor-to-background ratios of planar and SPECT imagings were 1.60 and 1.98 for 99mTc-tetrofosmin, 1.12 and 2.09 for 201Tl, and 1.19 and 1.80 for 99mTc-MIBI, respectively. In another patient 99mTc-tetrofosmin and 201Tl imagings were performed. Not only the primary tumor but also the direct invasions and metastatic lesions (bone metastases) were clearly detected. The tumor-to-background ratios of planar and SPECT imagings were 2.31 and 2.78 for 99mTc-tetrofosmin and 2.45 and 3.58 for 201Tl, respectively. In 99mTc-tetrofosmin scintigraphy we acquired delayed images, and the tumor-to-background ratios of planar and SPECT delayed images were 1.20 and 1.86, the retention ratios were -1.11 and -0.92 and the retention indices were -48.1 and -33.1, respectively. Our preliminary results suggest that 99mTc-tetrofosmin is useful in detecting not only the primary tumor but also metastatic lesions from malignant thymoma.- - - - - - - - - - ranking = 0.001709552596517keywords = bone (Clic here for more details about this article) |
10/100. Two cases of atypical carcinoid of the thymus.We describe two cases of atypical carcinoid of the thymus. One was an 82-year-old woman with complaints of nonproductive cough and back pain, and the other a 64-year-old woman with no symptoms. Computed tomography scans of the chest in both cases revealed a large mass in the anterior mediastinum. Multiple metastases to bone and liver were also noted in the former case. Histological examination of their tumors revealed that the tumor cells were arranged in a nested, trabecular, or pseudorosette pattern, with increased numbers of mitoses, nuclear pleomorphism, and presence of necrosis. In addition, immunohistochemically, the cells stained for neuron-specific enolase, synaptophysin and chromogranin a. Combination chemotherapy consisting of carboplatin and etoposide was performed as initial chemotherapy in the former case and as adjuvant therapy in the latter. The former patient achieved a short-term partial response. It is important to differentiate atypical carcinoid from other thymic tumors, since such tumors including thymoma have a much better prognosis than does atypical carcinoid.- - - - - - - - - - ranking = 0.001709552596517keywords = bone (Clic here for more details about this article) |
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