OBJECTIVES:Cervical rhabdomyosarcoma is extremely rare, and there is a paucity of literature on the subject. The purpose of this study was to describe the clinical and pathologic features of cervical rhabdomyosarcoma. METHODS:We retrospectively reviewed all patients with cervical rhabdomyosarcoma who presented to our institution from 1980 to 2010. We reviewed pathologic, demographic, and clinical information. RESULTS:During the study period, 11 females presented with cervical rhabdomyosarcoma. The median age at presentation was 18.4 years, and 6 patients were <19 years old at diagnosis. Vaginal bleeding was the most common presenting symptom, and a vaginal mass was often a co-presenting symptom. Eight patients (73%) presented with stage IB disease, and 8 (73%) presented with the embryonal (botryoid) histologic subtype. Nine patients (82%) received multimodal therapy consisting of surgery with chemotherapy, radiation therapy, or both. All patients were without evidence of disease after completion of primary therapy, but 3 patients experienced local recurrence. At a median follow-up of 23 months, 6 patients (55%) were without evidence of disease, 1 (9%) was alive with disease, 1 (9%) had died of disease, and 3 (27%) had died of other causes. Three patients (27%) had other primary malignancies in addition to rhabdomyosarcoma-1 had a Sertoli-Leydig tumor, 1 had a Sertoli-Leydig tumor and a pinealoblastoma, and 1 had thyroid cancer and a parotid adenocarcinoma. CONCLUSIONS:With multimodal therapy, cervical rhabdomyosarcoma appears to be associated with a good prognosis. Favorable prognostic factors such as early stage at diagnosis and a favorable histologic subtype may contribute to the excellent observed survival.

Objective To describe the clinicopathologic features and immunophenotypic characteristics in one cases of uterine tumors resembling ovarian sex cord tumor(UTROSCT).Methods The clinical findings,morphologic features and immunohistochemical markers in one case of UTROSCT were study.Results Sex cord patterns in UTROSCT contained epithelial-like cells and foam cells.The tumor cells expressed the most recently identified markers of sex cord differentiation(calretinin,Melan A,CD99,and inhibin) and smooth muscle(desmin and smooth muscle actin),with coexpression of cytokeratin,vimentin,estrogen receptor,progesterone receptor,and bcl-2 immunoreactivity,and negative for CK7,CD10 and CD117.Conclusion The morphologic and immunohistochemical findings in UTROSCT strongly support that these unusual uterine tumors are polyphenotypic neoplasms with true sex cord differentiation.

Between 1975 and 1989, 896 patients were treated for endometrial carcinoma at the Massachusetts General Hospital. Thirty patients were identified from the tumor registry as having uterine papillary serous carcinomas. The survival for all patients and for groups of patients stratified on clinical and pathological parameters was examined in the Kaplan-Meier survival curves. Curves for the different strata were compared using the logrank test. The 5-year survival for the 30 patients was 30% +/- 9%. Patients with surgical stage I and II tumors had a 5-year survival rate of 79% +/- 14% compared to 25% +/- 10% in patients with stage III and IV tumors (P = 0.02). Clinical stage, depth of myometrial invasion, lymph-vascular space invasion, tumor grade, and DNA aneuploidy were not found to significantly impact on survival. However, a survival advantage was seen in patients diagnosed with early surgical stage tumors, reinforcing the need for thorough staging at the time of laparotomy.

Presentation of CaseA 6 1/2-year-old girl was admitted to the hospital because of status epilepticus.The patient was the only child of a mother 30 years old at delivery and a father 35 years old. The child was delivered by cesarean section, reportedly because she was 31 days overdue. Her early growth and development were normal, and her immunizations were up to date. She was well until three weeks before entry, when a sore throat and headache developed, with a temperature of 37.2°C. Twenty-four hours later an erythematous rash with a texture of "fine sandpaper" was noted over the . . .

Presentation of CaseA 41-year-old woman was admitted to the hospital because of pain in the right lower abdominal quadrant.The patient was well until 36 hours earlier, when she experienced the onset of an insidious, throbbing "ache" in the right lower quadrant of the abdomen, accompanied by anorexia, nausea, and one episode of vomiting of a small amount of bilious liquid without blood. On the following day she consulted a physician, who prescribed bismuth subsalicylate and acetaminophen. The pain became more persistent, awakening the patient from sleep, and remained vaguely localized to the right lower abdominal quadrant. The patient . . .

Presentation of CaseA 43-year-old woman was admitted to the hospital because of low-back pain and a presacral mass.The patient was well until seven months earlier, when increasing pain developed in the left buttock; the pain was aggravated during her menses and by sitting and was improved on recumbency. A computed tomographic (CT) scan of the abdomen and pelvis, performed elsewhere, was reported to show a mass behind the uterus, just to the left of the midline, with extension around the rectum. An intravenous urographic examination was reported to show a pelvic mass with localized narrowing of the left . . .

To identify patterns of failure following curative resection of gastric carcinoma, the records of 130 patients undergoing resection with curative intent at the Massachusetts General Hospital were reviewed. The total local-regional failure rate was 38% (49/130 patients), with 21 patients having local-regional failure alone and 28 patients having local-regional failure and distant metastases. The incidence of local failure rose with the more advanced stages of disease. Tumors staged B2, B3, C2, and C3 had local-regional failure rates in excess of 35%. This group of patients might benefit from adjuvant radiation therapy to the tumor bed and regional lymphatics. Local-regional failure rate was highest in the anastomosis or stump 33/130 (25%), followed by the stomach bed 27/130 (21%). The overall incidence of distant metastases was 52% (67/130 patients) and rose in the more advanced disease stages. Tumors staged B2, B3, C2, and C3 had rates of distant metastases greater than 50%. Sixty-one patients (77%) had failure in the abdomen (liver, peritoneal surface, adrenal, kidney, and spleen, but excluding tumor bed, anastomosis, or regional nodes). Patients with Stage B2, B3, C2, and C3 tumors had total abdominal failure rates greater than 40%. The highest failure rates in the liver were in Stages B3 and C3, in which the subsequent development of liver metastasis was 40% and 47%, respectively. Peritoneal seeding occurred in 30/130 (23%) of patients and was highest in Stages C2 and C3, with rates of 27% and 41%, respectively.

Presentation of CaseA 40-year-old man was admitted to the hospital because of fever, headache, and a rash.The patient was well until two days earlier, near the end of a two-week visit to St. Thomas in the Virgin Islands, when he awoke with a pruritic, erythematous rash over the proximal portions of the arms and legs that soon spread to the trunk, sparing the face, palms, and soles. That evening he returned to Boston on schedule in an airliner and began to experience pain around his waist, a bitemporal headache with mild photophobia, and fever and chilliness while en . . .

Presentation of CaseA 24-year-old man was admitted to the hospital because of right flank pain and fever.The patient was a native of Haiti, who came to this country five years before admission to attend educational institutions. There was a history of "skin infections" of both legs in childhood that resulted in scarring. Two years before entry a tuberculin skin test was reported to be positive, and the patient used pills of an unknown type irregularly during the succeeding year; he was told that repeated x-ray films of the chest were normal. Fourteen months before entry the patient had . . .