Primary epithelioid angiosarcoma of the adrenal gland case report and review of the literature.

Primary angiosarcoma of the adrenal gland is extremely rare. Here, we report on a 70-year-old man with an angiosarcoma of the right adrenal gland who died 3 weeks after tumor resection due to intestinal infarction and acute renal failure. No metastases were found at autopsy. Histologically, the tumor showed a predominantly epithelioid differentiation. Immunohistochemical examination revealed positive reactivity for cytokeratin, epithelial membrane antigen, vimentin, factor VIII-related antigen, CD31, CD34 and Ulex europaeus agglutinin-I. Features of endothelial origin were also demonstrable by electron microscopy. The differential diagnosis of this uncommon neoplasm is discussed. The present case emphasizes problems in differential diagnosis that arise from its epithelioid differentiation. A review of the literature underlines the poor clinical outcome of adrenal angiosarcoma.     

Schwannoma with angiosarcoma. Report of a case and comparison with other types of nerve tumors with angiosarcoma

BACKGROUND: Schwannoma with angiosarcomatous change is a rare tumor, the clinical characteristics of which have not been analyzed. METHODS: A patient with schwannoma with angiosarcoma arising in the midneck and clinically mimicking a carotid body paraganglioma is described with a literature review of all previously reported cases and a comparison of their clinical features with those of schwannoma with conventional malignant transformation and cases of neurofibroma and malignant peripheral nerve sheath tumor (MPNST) with angiosarcoma. RESULTS: There are four reported cases, including the present case. Schwannoma with angiosarcoma affects older adults, mainly men. Three tumors arose from the vagus nerve in the neck. Three of the four angiosarcomas were epithelioid in type. Treatment in all cases was surgical resection followed by radiation and chemotherapy in one case and by radiation alone in another. One patient died with residual local angiosarcoma 5 months after the diagnosis. The remaining three patients were alive and disease free at 27 months, 43 months, and 90 months, with distant metastasis (after 15 months) reported only in the patient described in this case report. CONCLUSIONS: Schwannoma with angiosarcoma should be included in the differential diagnosis of presumed carotid body paragangliomas. Like angiosarcoma alone and schwannoma with conventional malignant transformation, but unlike cases of neurofibroma and MPNST with angiosarcoma, the patients are older adults, and there is a male prevalence. Schwannoma with angiosarcoma is capable of local spread with a fatal outcome and of distant metastasis, but follow-up strongly suggests that these patients have a better prognosis than patients with neurofibroma or MPNST with angiosarcoma. Recommended treatment is attempted complete surgical resection followed by radiation therapy and chemotherapy, if it can be tolerated by the patient.     

A case of intrahepatic cholangiocarcinoma with invasion to right adrenal gland

A 64-year-old woman was admitted for epigastralgia. Abdominal computed tomography demonstrated a large tumor in the right lobe of the liver to adrenal grand. An aspiration tumor biopsy was performed because of difficulty in diagnosing the tumor, and the pathological findings suggested intrahepatic cholangiocarcinoma. At surgery right hepatic lobectomy with caudate lobe combined with right adrenal glandectomy and diaphragmectomy was performed. Pathological findings showed moderately differentiated tubular adenocarcinoma with invasion to right adrenal gland, but no lymph node metastasis was involved nor invasion of lymph vessel and vein. The postoperative course was uneventful and the woman remains recurrence-free for 12 months after the surgery.     

Juvenile fibromatosis resembling aponeurotic fibroma and congenital multiple fibromatosis. One case with pleuropulmonary involvement

This article deals with a young school boy born in 1971 with a tumor in the palm of his right hand. We have considered this a juvenile aponeurotic fibroma. This tumor has recurred several times, leading to unavoidable amputation of the right wrist and hand. Seven years after the initial diagnosis, the axillary area became involved, then the pleura and lung. Such an unusual process leads to reconsidering the diagnosis and to regard this case as an intermediate form between Keasbey's juvenile aponeurotic fibroma and generalized fibromatosis.     

Treatment of desmoids and mesenteric fibromatosis in familial adenomatous polyposis with raloxifene

BACKGROUND: Among the great variety of extracolonic manifestations of familial adenomatous polyposis, the most serious are desmoids and fibromatosis of the abdominal cavity. These may be a danger to the patient and a concern to the clinician. Pharmacological management of this relentless problem is favored by surgical intervention. At present, however, beneficial actions of medical therapy are not separable from undesirable side effects. METHODS: We studied the effects of 120 mg daily of raloxifene, a non-steroidal benzothiophene, on progressive desmoid tumors and mesenteric fibromatosis by evaluation of lesion size and symptoms in 13 patients with familial adenomatous polyposis, selected on the basis of intra-abdominal localization of the lesion, on refractoriness to other medical treatments, and on estrogen receptor-alpha expression. RESULTS: The patients had a significant response to raloxifene therapy, with complete remission in 8 cases and partial response in 5 cases, evaluated by regression of symptoms and tumor size. Serum biochemical parameters did not show any significant changes. Side effects were never observed. CONCLUSIONS: Although the number of patients included in the study is limited and in spite of some limitations, the available results support that, in the evaluation of response, daily therapy with raloxifene decreases desmoid tumor and mesenteric fibromatosis size and symptoms and does not cause side effects. These findings offer a novel option in the pharmacological treatment of desmoids, leading to medical therapy of these neoplastic lesions in familial adenomatous polyposis patients.     

Congenital solitary fibromatosis of the skeleton: case report of a variant of congenital generalized fibromatosis

A study of a 3-month-old boy with a solitary form of congenital generalized fibromatosis of the skeleton is presented. Radiographic examination revealed a shortening of the right ulna and an osteolytic lesion in the distal metaphysis extending to the epiphysis. The histological appearance seems to be consistent with previously described cases of generalized congenital fibromatosis of soft tissues and skeleton. Ultrastructurally, the tumor cells resembled primitive fibroblasts. The lesion was curetted 3 times and filled with bone transplants before it finally healed. At follow-up of the boy at age 13, there were no signs of the tumor.     

原发性肺血管肉瘤1例报道

A 40-year-old man with haemoptysis and serious spontaneous hemothorax. Chest computed tomography showed multiple flakes in his lungs, nodules shadow, right pleural effusion with right pulmonary atelectasis. The pathological specimens obtained by thoracic surgery revealed pulmonary angiosarcoma. Primary pulmonary angiosarcoma is a rare malignant tumor and is lack of typical clinical symptoms and imaging features, diagnosis mainly depend on histopathologic features and immunohistochemical analysis. There is no effective therapy for pulmonary vascular sarcoma. Integrated treatment can may extend the survival time of patients.     

Cryosurgery of the adrenal gland

Adrenalectomy has become the standard of care for the management of hormonally active adrenal masses. Minimally invasive adrenal-sparing surgical techniques have recently been introduced for the treatment of benign adrenal lesions, with the intent of complete excision or destruction. Cryosurgery is one such modality that is focused on reducing patient morbidity and hastening postoperative recovery, while preserving normal tissue. The emerging interest in cryosurgery is attributable to improved delivery systems and advances in radiologic imaging. However, questions remain about the risks and benefits of this technology for adrenal-sparing surgery in terms of safety and effective tissue destruction. We examine our experience and discuss our results with open and laparoscopic adrenal cryosurgery.     

Dysgerminoma of the ovary with rhabdomyosarcoma. Report of a case

A 14-year-old girl with a large left ovarian mass underwent excision of the tumor which on histologic examination revealed dysgerminoma mixed with elements of rhabdomyosarcoma. A subsequent staging laparotomy revealed a solitary 1-cm nodule along the pelvic wall. This was composed of rhabdomyosarcoma. Review of literature revealed that soft tissue sarcomas of several types may on occasion be seen in association with gonadal and extragonadal mixed germ cell tumors or in spermatocytic seminoma of the testis. However, no previously published report of a sarcoma arising in a pure dysgerminoma was found in the literature.     

肠系膜纤维瘤病误诊为早期直肠癌根治术后复发1例报道及文献复习

肠系膜纤维瘤病是一种间叶组织来源的肿瘤,其病因不明、发病率低、无特异性表现、活检困难,因此临床定性诊断比较困难。本文介绍一例肠系膜纤维瘤病误诊为早期直肠癌术后复发的患者,并且通过相关文献复习,提高临床医生对本病的认识,从而减少误诊。     

Mesenteric fibromatosis in a patient with Camurati-Engelmann disease. A case report and literature review

Mesenteric fibromatosis is a rare type of desmoid tumor characterized by local aggressiveness and a tendency to relapse. In view of these characteristics it may be considered a low-grade fibrosarcoma. Camurati-Engelmann disease is a very rare form of bone dysplasia characterized by osteosclerosis of the diaphyses of the long bones. Here we describe the case of a male patient affected by these two rare diseases in association with chronic inflammatory intestinal disease.     

A case of fibromatosis of the breast

A 36-year-old woman presented with a 10 mm diameter mass in the right breast. Since the mass persisted for 3 months after detection and mammary carcinoma was suspected based on ultrasonographic findings, the mass was resected. Histologically, the mass demonstrated proliferative margins and consisted of spindle cells with bland cytologic features and abundant collagen. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin, and were negative for cytokeratins and desmin. Furthermore, the cells showed MIB-1 immunoreactivity with a MIB-1 labeling index of 4.1. Based on these findings, was diagnosed fibromatosis. Breast fibromatosis is rare and is usually misdiagnosed as breast carcinoma preoperatively. To date, only 10 cases of breast fibromatosis have been reported in Japan. Among the reported cases in Japan, our patient presented with the smallest mass, and ultrasonographic findings in this case were the same as those of other cases. Our experience and a review of the literature indicated that differentiation of fibromatosis from carcinoma is very difficult by ultrasonographic examination. In our case, despite involvement of the surgical margins, there was no recurrence. This may be attributed to the small size of the mass and focal exposure.     

Epithelioid cell's uterine leiomyoma uteri. A case report with immunohistochemical study

In the present report we refer to a rare case of uterine leiomyoma Uteri with epithelioid cells, characterized by an omogeneous light cell's structure and by a lymphangiomatous like histological aspect. An immunohistochemical study was carried out, using both endothelial and cell surface markers, as well as vascular markers. This study has allowed us to exclude the angiogenic origin of the neoplasm. It has, furthermore, revealed how the lymphangiomatous like histological aspect, even though being a peculiar component of the tumor, is to be put in relation with the epithelioid cells' layout, which has been evidenced by the immunohistochemistry to be of muscular nature.     

邻近腋窝的上皮样血管内皮瘤1例报道

We described a case of a 71-year-old woman with an epithelioid hemangioendothelioma (EHE) in her left axilla,a rare location which hasn't been reported yet. The patient suffered from numbness, pain and decreased muscle strength of her left upper extremity. Sonography revealed a hypoechoic mass surrounded the axillary artery and brachial artery. No obvious capsule was demonstrated. CT showed a soft-tissue mass with some calcifications and peripheral ring-like en-hancement. The MRI indicated a mass with mainly intermediate signal intensity on Tl-weighted imagine and intermediate signal intensity on T2-weighted imagine. The diagnosis was confirmed by histopathologic examination after surgery. There are some correlations of these imaging features with its histopathologic characters.     

Leiomyosarcoma of the adrenal gland and its angiographic features: A case report

A case of leiomyosarcoma of the adrenal gland is reported. This is a rare neoplasm at this location and actually may arise from the venous wall of the gland. Angiographically, this lesion is demonstrated as a vascular mass with early draining of the vein.     

High expression of neuropeptide y receptors in tumors of the human adrenal gland and extra-adrenal paraganglia.

PURPOSE: Recently, a role of neuropeptide Y (NPY) in tumor biology was suggested based on the high density of NPY receptors in breast and ovarian cancers. The high frequency of NPY receptors in steroid hormone-producing ovarian sex cord-stromal tumors, together with the known influence of NPY on steroid hormone and catecholamine secretion in the rodent adrenal gland, led to the investigation of NPY receptor expression in the human adrenal gland and related tumors. EXPERIMENTAL DESIGN: Fifteen adrenal cortical tumors, 20 paragangliomas, 23 pheochromocytomas, 20 neuroblastomas, and 8 normal adrenal glands were investigated by in vitro NPY receptor autoradiography using 125I-labeled peptide YY in competition experiments with receptor subtype selective analogs. RESULTS: Ninety three percent of cortical tumors express Y1, 35% of pheochromocytomas and 61% of paragangliomas express Y1 and Y2, and 90% of neuroblastomas express Y2 receptors. The NPY receptors in pheochromocytomas, paragangliomas, and neuroblastomas are often expressed concomitantly with the NPY hormone detected immunohistochemically. The adrenal cortex strongly expresses Y1, whereas no NPY receptors are found in the adrenal medulla. CONCLUSIONS: These receptor data suggest a role of NPY in adrenal cortical tumors and, together with the strong NPY innervation of the cortex, a physiologic role in the adrenal gland, mediated by Y1 receptors. These NPY receptors are a potential new molecular target for the therapy of malignant tumors.     

背部血管肉瘤1例

1 临床资料 患者,女,30岁,于2015年9月人院.约2岁时背部无明确诱因出现淤青色团块,后逐渐增大,无疼痛、出血等伴随症状,行激光治疗后好转.2013年患者原部位再次发现包块,为淤青色皮肤改变,其包块逐渐增大,高于皮肤并伴有疼痛.入院前2个月予平阳霉素注射及电频治疗,无好转,且包块持续增大伴疼痛加重.     

A case of angiosarcoma of the breast

This is a case report of a 20-year-old woman who had primary angiosarcoma of the left breast, with metastases to the spleen and ovary. Eight months after detecting a mass in her breast, she underwent mastectomy with biopsy of the ipsilateral axillary lymph nodes, splenectomy and bilateral oophorectomy. Five months after the operation, the patient succumbed to lung metastases. Angiosarcoma of the breast is a rare condition with a poor prognosis, and there are no established chemotherapeutic regimens as yet. Immunohistochemical staining for endoglin, known to be expressed mainly on the surface of endothelial cells, was positive. This suggests the possibility of treating angiosarcoma with anti-endoglin monoclonal antibodies.