1/13. Retrorectal cystic hamartoma: report of 5 cases with malignancy arising in 2.BACKGROUND: Retrorectal cystic hamartomas, or tailgut cysts, are rare congenital lesions that typically present as presacral masses. These lesions are frequently clinically unrecognized and misdiagnosed. Malignant change is extremely rare. Only 10 additional cases with associated malignancy were recovered from the literature. We describe the clinicopathologic features of 5 cases, including 2 cases with malignant transformation. RESULTS: All patients were women (age range, 36-69 years). The most common symptoms were pain with defecation and rectal bleeding. One patient was asymptomatic. All lesions presented as multicystic presacral masses and all were surgically resected. The lesions varied in size from approximately 2 to 12 cm (average, 9.5 cm) and overall had similar histology composed of a variety of epithelial linings (stratified squamous, transitional, and simple or ciliated pseudostratified columnar). skin adnexa, neural elements, and heterologous mesenchymal tissue, discriminators between retrorectal cystic hamartoma and teratoma, were not identified. Arising in association with the cysts was a focus of adenocarcinoma in one case and a neuroendocrine carcinoma in another. CONCLUSIONS: The clinical diagnoses in our cases were often delayed, which in part may be due to unfamiliarity with this entity. The main diagnostic difficulty is distinction from presacral mature cystic teratomas and rectal duplication cysts. Tailgut cysts require complete surgical excisions to prevent future recurrences and to preclude possible malignant transformation. Meticulous gross examination and adequate sampling are important to document the exact nature of these cysts and to rule out possible coexisting malignancies, which may be focal.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/13. Coexistence of metastatic neuroendocrine carcinoma of the uterine cervix with human immunodeficiency virus infection.women now constitute 28% of new cases of human immunodeficiency virus (hiv) infection. Cervical cancer in hiv-infected women has a high recurrence and death rate, as well as decreased intervals to recurrence and death. Neuroendocrine carcinomas of the cervix are characterized by a high frequency of early nodal and distant metastases. We present the first report of a neuroendocrine carcinoma of the cervix in an hiv-positive patient. A 28 year old with a 9-year history of hiv succumbed to metastatic neuroendocrine carcinoma of the cervix 5 months after diagnosis. Given the aggressive nature of the cell type, an extended metastatic workup should be considered prior to surgery. The immune suppression present in hiv-positive patients with neuroendocrine cervical carcinoma may make such a workup particularly crucial, such that surgery is offered only to those who can be expected to benefit.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/13. renin producing neuroendocrine pancreatic carcinoma--a case report and review of the literature.A 44 year old male patient presented with severe hypertension. The diagnostic work-up revealed elevated levels of plasma renin activity (about 10 times the upper limit of normal) in the presence of normal plasma aldosterone levels and serum potassium concentrations. Renovascular disease was excluded by angiography. Selective renal vein sampling did not show any renin gradient. CT-scans of the abdomen demonstrated normal morphology of the kidneys and adrenals but revealed a big mass in the pancreatic corpus and tail with infiltration of the splenic vein and the presence of enlarged local lymph nodes. The endocrine nature of the pancreatic mass was further supported by a positive octreotide scintigraphy scan. Surgical removal of the tumor by left sided pancreatectomy combined with splenectomy resulted in rapid normalization of elevated renin concentrations as well as blood pressure. Histological examination of the tumor tissue revealed the presence of a neuroendocrine pancreatic carcinoma. Highly (x 70) elevated renin levels were detected by radioimmunoassay in the tumor tissue.To our knowledge this is the first renin-producing neuroendocrine pancreatic carcinoma described in the literature. The present paper describes the case in detail and reviews the available literature on clinical symptomatology, diagnosis and treatment of renin-producing tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/13. Large cell neuroendocrine carcinoma of the uterine cervix: a clinicopathological study of six cases.Six cases of cervical large cell neuroendocrine carcinomas (LCNEC) were found among 972 patients (0.6%) with invasive cervical carcinoma. The patients, who were from 27 to 51 (mean 38) years of age, presented with vaginal bleeding or an abnormal Papanicolaou smear. Five tumors were stage Ib and one was IIa. All patients underwent radical hysterectomy and received adjuvant chemotherapy and pelvic radiotherapy. Four patients died of tumor 6 to 19 months (mean 14 months) postoperatively. On histologic examination, the tumor cells were arranged in an organoid growth pattern and were larger than those of typical small cell carcinoma. Glandular differentiation was present in one case. Mitotic figures ranged from 15 to 45 (mean 29) per 10 high-power fields. Prominent vascular invasion and necrosis was seen in all of the tumors. Each tumor was immunoreactive for chromogranin a and/or synaptophysin. The results of this study confirm the aggressive nature of cervical LCNECs. The recognition of LCNECs is necessary to establish the most effective treatment for these aggressive tumors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/13. Parapharyngeal space tumour presenting as recurrent uvular oedema.neoplasms of the parapharyngeal space are uncommon and usually present as an intra-oral or neck mass. They often elude early diagnosis due to their deep-seated nature. Here we report a case presenting with recurrent oedema of the uvula. The pathophysiology of this previously unreported mode of presentation is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/13. Multiple neuroendocrine tumors of the pancreas associated with pancreas divisum.pancreas divisum is the most common congenital anomaly of the pancreas, characterized by missing fusion of the ventral and dorsal pancreatic duct. It may cause pancreatitis, but is rarely associated with malignancy.We report herein for the first time the rare association, in a symptomless patient, of multiple neuroendocrine tumors of the pancreas with pancreas divisum and a failure of the exocrine system. Diagnosis was made incidentally by routine abdominal ultrasound. Laboratory examinations and a fine-needle aspiration revealed the neuroendocrine nature of the tumor. spleen-preserving left pancreas resection was performed, with evidence of multiple neuroendocrine tumors of the pancreas with the typical histological characteristics. Eighteen months later the patient is still free of tumor burden.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/13. Large cell neuroendocrine carcinoma of the larynx: a case report and a review of the classification of this neoplasm.This report describes a case of large cell neuroendocrine carcinoma (LCNEC) of the larynx. A 74 year old man who presented with otalgia underwent direct laryngoscopy with biopsy, which revealed an invasive poorly differentiated carcinoma. laryngectomy with bilateral neck dissections revealed invasion of the pre-epiglottic space by the tumour, with metastases to bilateral lymph nodes (AJCC T3N2c). The tumour was characterised by large cells with vesicular chromatin and prominent nucleoli. The cells were arranged in organoid and trabecular patterns with a background of extensive necrosis and numerous mitotic figures. Immunohistochemical and ultrastructural analyses confirmed the neuroendocrine nature of the tumour. Metastatic disease was present in the liver, and the patient died within weeks of surgery. LCNEC carcinoma is a rare tumour of the larynx. Recognition at this site is essential so that proper patient management can be initiated.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/13. Metastatic thymic carcinoid: a case report.Thymic neuroendocrine carcinomas (carcinoid) are rare tumors. They have malignant potential, the capacity for distant metastasis, and often present with associated endocrinopathies. This report describes a patient who was diagnosed with thymic carcinoid and cushing syndrome at age 19 that, despite complete surgical excision of his tumor, developed local recurrence with distant metastases to his brain, lungs, and bone. We discuss the evolution of this patient's illness as well as the therapies used in his care. Due to the nature of these tumors to recur both locally and distant, the importance of aggressive surgical management is emphasized. We also discuss the role of adjuvant therapy, which in our case consisted of chemotherapy, radiotherapy, and several new therapies including an antiangiogenesis agent and a tyrosine kinase inhibitor.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/13. neuroendocrine tumors of the liver and pancreas associated with elevated serum prostatic acid phosphatase.A 58-year-old man was revealed to have multiple liver tumors with elevated prostatic acid phosphatase (PAP) during a medical examination. The tumors were of neuroendocrine nature, but no abnormal findings were obtained in other organs in which neuroendocrine tumors develop frequently. Repeated transarterial embolization was partially effective. However, the tumors became resistant to the therapy three years later, continued growing and ruptured. autopsy disclosed neuroendocrine tumors in the pancreas, which were immunohistologically positive for PAP. neuroendocrine tumors of the pancreas and liver producing PAP are rare; this case is reported with a review of literature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/13. Retroperitoneal cystic neuroendocrine tumor. A case report.A 21 cm retroperitoneal cystic mass was excised from a 71 year old woman. The cyst was filled with a hemorrhagic fluid and contained a 5 cm parietal hemorrhagic nodule. On histology, the nodule was composed of a uniform population of round cells arranged in trabeculae and nests. The neoplastic cells were immunoreactive to cytokeratin, EMA, NSE, chromogranin a, pancreatic polypeptide (PP) and Gastrin (G). Ultrastructural observation of neurosecretory granules confirmed the neuroendocrine nature of the tumor. No other lesions were detected and a diagnosis of primary epithelial neuroendocrine tumor was rendered. The histogenesis of the tumor including the possibility of a paraganglionic origin is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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