1/111. Disseminated cystic lymphangiomatosis presenting with acute abdomen: report of a case and review of the literature.lymphangioma is an uncommon tumor. Lymphangiomatosis, a benign tumor consisting of a cluster of dilated lymphatic channels, is very unusual. Most lymphangiomatoses are found in the neck and head area. Less than 5% are diagnosed intraabdominally and they are very infrequently encountered in the retroperitoneal area. Herein, we report a rare case of a 32 year-old woman who had disseminated intra-abdominal and retroperitoneal cystic lymphangiomatosis, which presented as acute abdomen. She received exploratory laparotomy due to the suspicion of malignancy, which was finally confirmed as cystic lymphangiomatosis. The clinical manifestations, imaging features, and management of this patient are discussed and compared with previous literature.- - - - - - - - - - ranking = 1keywords = abdomen (Clic here for more details about this article) |
2/111. Intra-abdominal embryonal rhabdomyosarcoma in an adult.rhabdomyosarcoma is an uncommon neoplasm in the adult population. Sporadic cases of primary rhabdomyosarcoma arising in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. We report a well-documented case of primary intra-abdominal rhabdomyosarcoma in a 57-year-old woman. The patient presented with a pelvic mass and an elevated serum CA 125 and was referred to gynecologic oncologists at our institution for a presumed primary gynecologic malignancy. Intraoperatively, amorphous gelatinous tumor comprised a large portion of the peritoneal cavity. Surgical exploration of the abdomen failed to implicate any specific organ as the site of origin of the tumor. The overall histologic pattern of the resected tumor was most consistent with embryonal type rhabdomyosarcoma. To our knowledge this is the first well-documented case report of non-hepatobiliary, adult, intra-abdominal embryonal rhabdomyosarcoma in the English language literature. The presentation of a rare adult sarcoma mimicking a gynecologic malignancy was an unusual feature that complicated the diagnosis in this case.- - - - - - - - - - ranking = 0.4keywords = abdomen (Clic here for more details about this article) |
3/111. Desmoplastic small round-cell tumor of the paratesticular region: report of an adult case with demonstration of EWS and WT1 gene fusion using paraffin-embedded tissue.Desmoplastic small round-cell tumor typically occurs in the abdomen of young men, but it can also develop at other anatomic sites and in older people, presenting greater diagnostic difficulties. We report a case of this tumor arising from the paratesticular region in a 43-year-old man. The tumor showed histologic, immunohistochemical, and ultrastructural evidence of multilineage differentiation, including epithelial, mesenchymal, and neuronal features. In addition, the presence of an EWS and WT1 chimeric messenger rna was demonstrated by the reverse transcriptase-polymerase chain reaction using an EWS exon 7 primer and WT1 exon 8 and exon 9 primers, which revealed single polymerase chain reaction products with a junction of EWS exon 7 to WT1 exon 8. Our study demonstrates that desmoplastic small round-cell tumors of the paratesticular region share not only morphologic but also molecular genetic features with those of the abdomen and that reverse transcriptase-polymerase chain reaction analysis using paraffin sections is useful for a conclusive diagnosis.- - - - - - - - - - ranking = 0.4keywords = abdomen (Clic here for more details about this article) |
4/111. Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature.Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.- - - - - - - - - - ranking = 0.2keywords = abdomen (Clic here for more details about this article) |
5/111. Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: report of a case.tuberous sclerosis is a hereditary autosomal-dominant disease characterized by hamartomas that can develop in any organ. We report herein the case of a 34-year-old female with tuberous sclerosis and a huge abdominopelvic mass that started growing quickly 2 years after its diagnosis. The patient had undergone several previous operations for hydrocephalus and cerebral tubers, and a nephrectomy for right renal angiomyolipoma. On admission, she was in poor general health with renal failure, severe anemia, and weight loss. A laparotomy revealed that the tumor occupied the pelvis, the lower and part of the upper abdomen, and was hypervascularized, with an extremely irregular surface covered in nodules, vegetations, and areas of hemorrhagic necrosis. The development of the mass and the impossibility of recognizing the internal genital organs led us to assume that the formation had originated from these. Frozen-section examination indicated an undifferentiated tumor that had not been completely resected. Her postoperative course was complicated by bronchopneumonia and progressive renal failure. The patient died 10 days after surgery due to cardiorespiratory failure. A histological diagnosis of epithelioid angiomyolipoma was confirmed. Although it is presently impossible to determine whether angiomyolipoma with predominant epithelioid cells is more aggressive than typical angiomyolipoma, it definitively demonstrated local aggressive behavior in this patient.- - - - - - - - - - ranking = 0.2keywords = abdomen (Clic here for more details about this article) |
6/111. A myxoid liposarcoma in the lower leg, with a large intra-abdominal metastasis.We report a patient with a large intra-abdominal metastasis of myxoid liposarcoma. The patient first noticed an asymptomatic mass in her left leg in 1985, when she was 20 years old. The mass was left untouched until she realized its rapid growth and consulted a local doctor in 1994. After needle biopsy, she was histologically diagnosed as having a myxoid liposarcoma. She disagreed with the recommendation for an amputation below the knee, made at another hospital. A marginal resection was performed as an alternative treatment. She subsequently underwent three more marginal resections and four intra-lesional resections for repeated local recurrences. In 1997, an abdominal computed tomography scan revealed the presence of multiple intra-abdominal metastases, and the lesions were judged to be inoperable. ileus and respiratory distress, caused by compression by the abdominal mass, gradually worsened, and she died in 1999, at the age of 34. The girth of her abdomen was 135 cm at the time of death.- - - - - - - - - - ranking = 0.2keywords = abdomen (Clic here for more details about this article) |
7/111. Endometrial stromal sarcoma: objective response to letrozole.BACKGROUND: Low-grade endometrial stromal sarcoma is generally an indolent tumor rich in estrogen and progesterone receptors. Objective responses to hormonal therapy, most commonly with megestrol acetate, have been reported. CASE: The patient is a 51-year-old woman who presented with low-grade endometrial stromal sarcoma confined to the uterus in 1991 and was treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Approximately 5 years later, the patient had recurrent pelvic disease treated with radiation therapy, followed by an attempt at resection. She was treated with megestrol acetate during the period she received radiation therapy with poor tolerance. tamoxifen was then given with no tumor response. megestrol acetate was restarted with progression of disease in the pelvis and abdomen. Letrozole was then given at a daily dose of 2.5 mg with partial response for a duration of 9 months. CONCLUSION: Letrozole at a daily dose of 2.5 mg may be effective in low-grade endometrial stromal sarcoma with positive estrogen receptors.- - - - - - - - - - ranking = 0.2keywords = abdomen (Clic here for more details about this article) |
8/111. A case of secondary myocardial lymphoma presenting with ventricular tachycardia.Malignant lymphoma can involve the cardiac cavity or myocardium as a mass. Clinical symptoms of its cardiac involvement are usually absent or nonspecific, making the diagnosis of the cardiac involvement very difficult before death. We experienced a patient with secondary myocardial non-Hodgkin's lymphoma presenting with sustained ventricular tachycardia (VT) as a primary clinical problem. A 39-yr-old woman visited our hospital because of dyspnea and palpitation for 7 days. physical examination revealed rapid heart beat with variable intensity of the first heart sound and soft mass in the lower abdomen. VT with a cycle length of 480 msec was recorded in resting 12-lead electrocardiogram. Two well-circumscribed hypo-echogenic round masses were demonstrated in the interventricular septum and left ventricular posterior wall. Cytological examination of aspirated pericardial fluid and percutaneous needle biopsy of the abdominal mass revealed a diffuse large cell type non-Hodgkin's lymphoma. Myocardial masses and ventricular tachycardia resolved with chemotherapy using cyclophosphamide, adriamycin, vincristine and prednisone regimen. To our best knowledge, the same case as ours has not been reported previously.- - - - - - - - - - ranking = 0.2keywords = abdomen (Clic here for more details about this article) |
9/111. Sporadic intra-abdominal desmoid with acute abdomen.We report a 72-year-old man with sporadic intra-abdominal desmoid tumor manifesting as acute abdomen. CT scan revealed an air-containing tumor 7 cm in diameter; three weeks later, the tumor had shrunk to 4 cm on antibiotics. At surgery, a tumor arising from the transverse colon mesentery and infiltrating the jejunum was resected. No recurrence occurred over a 1-year follow-up.- - - - - - - - - - ranking = 1keywords = abdomen (Clic here for more details about this article) |
10/111. Desmoplastic small round cell tumour: a description of two cases and review of the literature.BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a recently described neoplasm, typically occurring in adolescent and young males. It usually shows an aggressive behaviour, presents in the abdomen, often with diffuse peritoneal implants. It has been demonstrated to be a chemosensitive tumour, generally with short-lasting response and poor survival gain from systemic chemotherapy. The authors report two additional cases of DSRCT and review the available medical literature. patients AND methods: Two young males with intra-abdominal DSRCT were treated with a first-line chemotherapy including carboplatin, doxorubicin and etoposide. Results: Both of the patients obtained a partial response after first-line chemotherapy. The first patient started, subsequently, CD34 stem cell mobilisation with high-dose cyclophosphamide (7 g/m(2)) in order to perform high-dose chemotherapy, but CD34 cell count was insufficient to practice leukapheresis; he died 34 months after the diagnosis because of progression of the disease. The second patient underwent cytoreductive surgery, but progressed 2 months later despite second-line treatment; he died 16 months after the diagnosis. CONCLUSION: This experience confirms that DSRCT may be considered a chemosensitive tumour, highly aggressive, with short-lasting response to chemotherapy. Anyway, the recent literature suggests that multidisciplinary treatment including chemotherapy, surgery and radiation might be the proper approach to this rare malignancy.- - - - - - - - - - ranking = 0.2keywords = abdomen (Clic here for more details about this article) |
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