1/71. Epithelioid hemangioendothelioma of the greater omentum: report of a case.We report herein the case of an 11-year-old girl found to have an epithelioid hemangioendothelioma of the greater omentum. The child presented with a 4-month history of vague lower abdominal pain and gradually increasing generalized distension. Her symptoms and signs could not be ascribed to any specific organ system. ultrasonography and computerized axial tomography revealed a mass lesion anterior to the bowel loops and a laparotomy revealed a huge lobulated mass, arising from the greater omentum, which was completely excised. She has remained well postoperatively without any adjunctive chemotherapy or radiotherapy.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/71. Mesotheliomas with deciduoid morphology: a morphologic spectrum and a variant not confined to young females.Deciduoid mesotheliomas are rare with only four previously reported cases, all affecting the peritoneum of young females. We describe another six cases (three men and three women; age range 52-65 yrs, median 55 yrs; five peritoneal and one pleural). Three patients had an occupational history of asbestos exposure. The deciduoid appearance predominated in four cases, whereas in two it represented a minor component within conventional tubulopapillary epithelioid mesothelioma. All tumors were strongly cytokeratin-positive (including CK5/6) and all showed at least focal staining for thrombomodulin, HBME-1, and calretinin. All were negative for epithelial mucin (D/PAS), CEA, BerEP4, LeuM1 (CD15), CD21, CD35, and S100 protein. Five of six cases (83%) were vimentin-positive and two (33%) were focally positive for alpha-smooth muscle actin. A differential diagnosis of gastrointestinal autonomic nerve tumor (GANT) had been initially considered from the morphology of one case, and we found positivity for some of the "neuronal" markers described in GANTs. This prompted us to apply such a panel to the other five tumors, accepting that the cytokeratin positivity encountered in all of our cases would exclude GANT. All cases of deciduoid mesothelioma (100%) were positive for PGP 9.5 and NSE and four of six (66%) were positive for NKI/C3. Weak focal staining (<5% cells) for synaptophysin was seen in two of six tumors. All cases were chromogranin-negative. All cases examined by electron microscopy showed desmosomes and smooth microvilli without rootlets but no neuroendocrine granules. In conclusion, a deciduoid morphology appears to be part of the histopathologic spectrum encountered in epithelioid mesothelioma. This variant is not confined to female patients and occurs over a wider age range than previously recognized. The overlapping immunophenotype with GANTs illustrates that caution should be exercised when interpreting positivity for "neuronal" markers in this context. An immunohistochemical panel that includes cytokeratins should always be used.- - - - - - - - - - ranking = 0.17923947627585keywords = nerve (Clic here for more details about this article) |
3/71. Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature.BACKGROUND: Only ten cases of the rare occurrence of a malignant peripheral nerve-sheath tumor (MPNST) arising in a ganglioneuroma either de novo or at a site of previous irradiation have been reported. patients AND methods: We present two children who at the age of 19 months and 6 months were diagnosed with a cervicothoracic ganglioneuroblastoma and a retroperitoneal neuroblastoma, respectively. They both received radiation therapy as part of the treatment of their disease. RESULTS: Following a 12-year interval, MPNST arose inside a benign ganglioneuroma in both patients. We illustrate the imaging findings in these two cases and review the cases reported in the literature to increase awareness of this association among radiologists.- - - - - - - - - - ranking = 0.89619738137926keywords = nerve (Clic here for more details about this article) |
4/71. Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.- - - - - - - - - - ranking = 0.17923947627585keywords = nerve (Clic here for more details about this article) |
5/71. Biphasic intra-abdominal desmoplastic small cell tumor in a patient with proximal spinal muscular atrophy.A case is reported of intra-abdominal desmoplastic small cell tumor (IDSCT) with biphasic histologic features in a patient with proximal spinal muscular atrophy. The tumor was composed of small epithelial cell nests with spindle cell sarcomatous areas. Both areas were surrounded by a desmoplastic stroma. Immunohistochemical studies revealed reactivity for low molecular weight cytokeratin, epithelial membrane antigen, vimentin, desmin and Leu-7 in both areas. Electron microscopic examination demonstrated paranuclear aggregates of intermediate filaments, zonula adherens and basement membrane-like material in the epithelial cells, while spindle cells in the tumor had fewer intracytoplasmic organelles. However, intermediate or transitional forms of both types of tumor cells were frequently observed. Although IDSCT are known to express multi-phenotypes immunohistochemically, attention should be paid to the broad spectrum of cell morphology in these tumors.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
6/71. Extrauterine Mullerian adenosarcoma of the peritoneum with an extensive rhabdomyosarcomatous element and a marked myxoid change.A case of extrauterine Mullerian adenosarcoma of the peritoneum in a 20-year-old woman is reported. The tumor was widely based on the abdominopelvic wall and there were no unusual features in the genital organs. The cut surface of the tumor showed a marked gelatinous appearance. The tumor was composed of an admixture of benign Mullerian-type epithelium and sarcomatous stroma. The predominant element of the sarcomatous area was rhabdomyosarcoma, which showed a close resemblance to well-differentiated embryonal rhabdomyosarcoma. In another sarcomatous area, fibroblastic cells without myoblastic properties diffusely proliferated in a marked myxoid background with some collagen bundles. Both the mitotic count and Ki-67 proliferative index of these cells were lower than those of rhabdomyoblastic cells. On follow up, the patient was disease free for 1 year postoperatively, without any subsequent treatment. The present case indicates that extrauterine adenosarcoma can also show histological heterogeneity as do uterine adenosarcomas. The remarkable myxoid change of this tumor seemed to be more largely due to a fibromyxoid element than a rhabdomyosarcomatous element, and the coexistence of the former may be related to the less aggressive behavior of this tumor.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
7/71. Primary peritoneal carcinoma presenting on routine papanicolaou smear.BACKGROUND: Primary peritoneal carcinoma is an uncommon disease, characterized by peritoneal carcinomatosis without other identifiable primary tumor. It typically presents resembling ovarian cancer, with abdominal pain and distention and in an advanced stage. We report a unique presentation of this disease. CASE: A 76-year-old woman had severe glandular dysplasia on a screening Papanicolaou smear. An ectocervical lesion was biopsied, revealing moderately differentiated adenocarcinoma. Cervical stenosis prevented sampling of the endocervix and endometrium. colonoscopy and mammography did not reveal malignancy. Total abdominal hysterectomy with bilateral salpingo-oophrectomy was performed. Surgery revealed surface implants on the pelvic organs, with minimal involvement of the ovaries. Histologic examination revealed adenocarcinoma with papillary serous differentiation. Surgical and microscopic findings were consistent with a diagnosis of primary peritoneal carcinoma. CONCLUSION: Primary peritoneal carcinoma usually presents in a manner similar to that of ovarian cancer, but atypical presentations also occur. While a Papanicolaou smear suggestive of carcinoma usually represents a primary cervical malignancy, this case serves as a reminder that other metastatic malignancies should be considered.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
8/71. Successful surgical treatment for implanted intraperitoneal metastases of hepatocellular carcinoma.We report here two patients with hepatocellular carcinoma who experienced implanted metastases in the abdominal cavity after hepatectomy or microwave coagulo-necrotic therapy. Hepatic resection and microwave coagulo-necrotic therapy were successful for these tumors, and the postoperative status was satisfactory in both patients. Implanted metastases were discovered in the abdominal cavity of each of these two patients 6 months after surgery. It is necessary to look not only for the presence of liver metastasis but also for the recurrence of the tumor in the abdominal cavity during the follow-up period. Generally, surgical resection for intraabdominal implanted tumors arising from any other abdominal organs is not indicated for improving the patient's quality of life. However, resection of metastatic tumors that occur in the abdominal cavity, arising from hepatocellular carcinoma may be of value in improving patient survival.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
9/71. A rare case of pseudomyxoma peritonei presenting an unusual inguinal hernia and splenic metastasis.pseudomyxoma peritonei (PMP) is a rare clinical entity in which a diffuse collection of intraperitoneal gelatinous fluid is associated with gelatinous implants on the peritoneal surfaces and omentum. Hematogenic or lymphatic metastasis is extremely rare. In addition, an inguinal mass as an initial presentation is also relatively rare. This is a case report of a PMP patient who had splenic metastasis and showed an inguinal tumor as an initial presentation. A 59-year-old female patient, who had undergone bilateral oophorectomy because of a ruptured ovarian mucinous tumor of boderline malignancy 12 years previously, presented a presumptive diagnosis of a left inguinal irreducible hernia. Computed tomography revealed a low density mass in the pelvic cavity and in the inguinal lesion, as well as in the spleen without any diseases around the organ. The preoperative serum carcinoembryonic antigen (CEA) level was elevated. The patient underwent a resection of gelatinous tumor in the pelvic cavity, splenectomy, and appendectomy, as well as left inguinal herniorrhaphy. Histological examinations revealed a splenic metastasis of PMP originating from the ovarian low-grade mucinous tumor. She received postoperative intraperitoneal lavage as well as chemotherapy, and has survived for over 7 years postoperatively without any evidence of recurrence, as confirmed by repeated follow-up CT examinations and CEA determination. Splenic metastasis of PMP is extremely rare; this represents only the third reported case of its kind in the literature. Furthermore, it should be noted that an inguinal tumor can sometimes be an initial presentation of PMP.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
10/71. Acute abdomen due to torsion of a pedunculated mesenteric fibroma.A case history of a boy with an acute abdomen due to torsion of a pedunculated mesenteric fibroma is presented. A review of the literature shows that only a relatively small number of mesenteric fibromata have been reported. In these cases the tumor was described as growing between the two leaves of the mesentery. The symptoms these tumors gave usually were due to the size of the tumor and compression of adjacent organs. The present case is unusual because of the pedunculated nature of the fibroma and its presentation as an acute abdomen.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
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