1/11. Neuroendocrine small cell carcinoma of the uterine cervix: what disease? What treatment? Report of ten cases and a review of the literature.Neuroendocrine small cell carcinoma of the uterine cervix (NESCCC) is an entity with very aggressive behaviour. The optimal initial therapeutic approach to this rare disease has not yet been clearly defined. We reviewed our experience of this disease over the past 10 years with regard to chemosensitivity. Since 1988, ten patients (mean age 33 years; range 24-47) have been diagnosed with NESCCC and treated in our institutions. Disease stage at presentation was IA (one), IB (five, two with lymph node involvement), IIB (one), IIIB (one), and IV (two). One patient had metastatic disease at presentation; three developed metastases during initial treatment. Eight patients underwent surgery and eight received radiation therapy. Six patients received pre- or postoperative cisplatinumvepeside (PE) combination chemotherapy, either alone or concurrently with radiation therapy. PE alone as primary chemotherapy led to disease stabilization in the two patients so treated; concurrent PE and radiation therapy resulted in a pathological complete response in one patient. Eight patients relapsed within 16 months and died of their disease within 29 months from the initial diagnosis. Two patients are alive with no evidence of disease at 13 and 53 months. Our series confirms the previously described very poor prognosis of NESCCC, despite initial aggressive multidisciplinary treatment. It may be that the introduction of chemotherapy, especially combined primary chemoradiotherapy, might allow patients to do a little better, although at the moment there is no good evidence one way or the other.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/11. Pleural small cell carcinoma in pre-existent asbestos related pleural disease.Extrapulmonary small cell carcinoma (SCC) is a very rare disease, and a primary pleural manifestation is extremely rare. A case of SCC of the pleura in a 66-year-old man with pre-existent asbestos-related pleural plaques is presented. This is the first case of pleural SCC in a patient with asbestos-induced pleural disease and the third reported case of a pleural SCC. The SCC developed in an area with pre-existent pleural thickening, underlining the need for careful analysis of alterations in the manifestation of pleural disease in patients with asbestos exposition.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/11. Small cell carcinoma of the endometrium with concomitant pelvic inflammatory disease.BACKGROUND: Small cell carcinoma of the endometrium is a rare disease entity characterized by bulkiness and predisposition to necrosis. Clinical presentations include postmenopausal bleeding, lower abdominal mass, chronic abdominal pain and menorrhagia. We present a case of small cell carcinoma of the endometrium with concomitant pelvic inflammatory disease. The literature is also reviewed. CASE: A 64 year old female presented was admitted with the principal complaints of fever, lower abdominal pain and malodorous vaginal discharge. Bimanual examination revealed cervical motion tenderness with a WBC of 9400 cells/microL and increased levels of neutrophils, band cells and c-reactive protein. Sonography revealed an adnexal echocomplex compatible with tubo-ovarian abscess. culture of the vaginal discharge revealed the presence of E. coli. Symptoms persisted despite three days of antibiotics administration so a laparotomy was performed with a friable hemorrhagic uterus revealed and an area of necrosis evident in the left adnexa. Malignancy was confirmed from frozen section. Total abdominal hysterectomy, with bilateral salpingooophorectomy and optimal debulking, was performed. The final pathology report confirmed small cell carcinoma of the endometrium. CONCLUSION: Malignancy and pelvic inflammatory disease have overlapping clinical characteristics. Once pelvic inflammatory disease is suspected in a postmenopausal patient, malignancy should also be suspected, and a thorough examination and a tumor-marker analysis performed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/11. Small cell undifferentiated carcinoma of the pancreas. Report of a patient with tumor marker studies.BACKGROUND. Small cell undifferentiated carcinoma of the pancreas is a rare type of pancreatic neoplasm. methods. The authors report the clinical and pathologic aspects of a patient with this malignant lesion and an extensive serologic and immunohistochemical survey of potential ectopically produced hormones or tumor markers. RESULTS. Neuron-specific enolase (NSE) emerged as a tumor marker. CONCLUSIONS. NSE could be valuable in the diagnosis and management of other patients with this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/11. Extraosseous accumulation of 99mTc-MDP in lymph node metastases of small cell carcinoma of the esophagus.We report a case of esophageal carcinoma that showed extraosseous accumulation of 99mTc-MDP in lymph node metastases to the cervical and paracardial lymph nodes. There are few cases showing abnormal extraosseous accumulation of 99mTc-MDP in esophageal cancer lesion. The patient was a 53-year-old man with advanced esophageal cancer. Bone scintigraphy demonstrated extraosseous accumulations in left supraclavicular and paracardial lymph node metastases. The histopathological diagnosis was small cell carcinoma of the esophagus, which is a rare disease with aggressive behavior and poor prognosis. Our patient underwent 2 courses of systemic chemotherapy (CDDP VP16), but died of rapidly growing systemic metastases 5 months after the initial treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/11. Curative resection of a small cell carcinoma of the pancreas: report of a case of long survival without chemotherapy.A 41-year-old asymptomatic man was, by chance, diagnosed as having a mass in the left upper quadrant of the abdomen by ultrasound. Computed tomography and magnetic resonance imaging showed a slightly enhanced heterogeneous mass, measuring about 5 cm in diameter, adjacent to the pancreas tail and spleen. On abdominal angiography the tumor was found to be fed by the splenic artery, and no encasement was observed. At operation the tumor was connected to the pancreas tail and attached to the spleen, and no metastasis was evident. As a result of these observations, a curative resection was performed. On histological examination the tumor cells had no ductal or architectural organization and were continuous to the normal pancreatic tissues. The cells were negative for Grimelius argylphile and the periodic acid schiff stain. immunohistochemistry indicated that the tumor cells were positive for keratin and epithelial membrane antigen, but negative for chromogranin a, vimentin, alpha1-antitrypsin and alpha1-antichymotrypsin. The tumor was diagnosed as a small cell carcinoma of the pancreas, which is a rare disease. In previously reported cases curative resection of the tumor had not been possible because of its rapid progression and metastasis, and the prognosis is generally very poor. In our case, however, curative resection of the tumor was done, and the patient is healthy with no signs of recurrence for 56 months after the operation and with no additional therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/11. Neutropenic enterocolitis in adults: case series and review of the literature.Necrotizing enterocolitis in adults is a rare disease and, in the past, has been associated with nearly uniform mortality. In recent years, necrotizing enterocolitis, now termed neutropenic enterocolitis, in adults has become more prevalent as a complication of aggressive systemic chemotherapy. In this report, we discuss two cases of neutropenic enterocolitis secondary to the administration of systemic chemotherapy in adult cancer patients: one with lung carcinoma, the other with leukemia. Both patients were successfully treated with early surgical intervention for resection of all necrotizing enteric lesions, and subsequent aggressive critical care support. Our experience suggests that early surgical intervention in adult patients with intestinal necrosis due to chemotherapy is essential to avoid mortality from this condition. Given the widespread, aggressive use of systemic chemotherapy in the neoadjuvant setting, patients at risk for this potentially lethal complication of neutropenic enterocolitis are increasingly common.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/11. Changes to levels of serum neuron-specific enolase in a patient with small cell carcinoma of the pancreas.Small cell carcinoma (SCC) of the pancreas is a rare disease, with an extremely poor prognosis; only 24 cases have been reported in the literature. However, as some patients have been successfully treated with combination chemotherapy, it is important to obtain both a definite diagnosis and a precise evaluation of the effect of the treatment. A 69-year-old woman presented with an abdominal tumor and pain. She had been observed for sensory neuropathy and swelling of the pancreatic head by the referring doctor over the previous 9 months. The patient was diagnosed with SCC of the pancreas after surgery and had two courses of combination chemotherapy (cisplatin and etoposide). Initially, the tumor disappeared completely on computed tomography (CT) scans, but she died of disease recurrence 3 months after completing the chemotherapy. Changes in serum neuron-specific enolase (NSE) levels were monitored constantly during the progress of the disease. NSE levels had already increased above the upper limit of normal 8 months before the patient's admission to our hospital, and levels changed concurrently not only with tumor growth but also subsequently with remission and then relapse of the disease after treatment. These results indicate that NSE is a good marker, both as a diagnostic indicator for SCC of the pancreas and as a means of evaluating response to treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/11. Primary gastric small cell carcinoma: report of a case and review of the literature.A 52-year-old man suffering from a pure-type primary gastric small cell carcinoma was treated with surgery and combination chemotherapy. The small cell carcinoma, approximately 6.5 cm in diameter, was situated in the posterior wall of the antrum and there were no distant metastases. Total gastrectomy and regional lymph node dissection was carried out. Histological examination revealed a solid pattern of proliferation of small cells with hyperchromatic, round nuclei and scant cytoplasm. Neoplastic cells infiltrated into the subserosal layer with severe lymphatic and vascular invasion. Regional lymph node cells were mostly replaced by tumor cells that stained positive for Grimelius, neuron-specific enolase (NSE), and synaptophysin. Accumulations of electron-dense core granules in the small neoplastic cells were seen by electron microscopy. Following surgery, the patient was treated with adjuvant chemotherapy consisting of cisplatin and etoposide. The patient is alive and recurrence free 3 years after surgical operation. We review 107 published cases of primary gastric small cell carcinoma, an extremely rare disease first reported in 1976. Small cell carcinoma is an aggressive, malignant tumor. Intensive chemotherapy is essential for patient survival even when curative surgical resection is carried out.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/11. A case of small cell carcinoma of the stomach.A case of small cell carcinoma of the stomach is reported. A 53-year-old male was referred to our hospital for elective surgery for gastric cancer. Pre-operative examinations revealed no metastases. gastrectomy was performed curatively, and there were no gross findings of metastases. Histologically, the tumor was composed of intermediate-sized cells with hyper-chromatic nuclei and scanty cytoplasm. These cells were argyrophilic and positive for chromogranin a. A small portion of the tumor consisted of conventional adenocarcinoma (signet ring cell carcinoma and tubular adenocarcinoma). No lymph node metastasis was observed microscopically. However, 7 months after the operation, splenic and hepatic metastases were detected, and the patient died very soon thereafter. Small cell carcinoma of the stomach is a very rare disease. In literature, only 15 cases have been cured surgically. Among them, only one case had been diagnosed as small cell carcinoma before the operation, which suggests the difficulty of pre-operative diagnosis. The prognosis of this disease is very poor compared with the common type of gastric carcinoma. Considering the poor prognosis of this particular disease, adjuvant chemotherapy might be mandatory in all cases even if surgically curative resection is performed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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