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.     

HIF transcription factor expression and induction of hypoxic response genes in a retroperitoneal angiosarcoma

Angiosarcoma is a rare and highly aggressive tumor of endothelial origin. The molecular mechanisms driving angiosarcoma growth have not been fully elucidated, although autocrine stimulation by vascular endothelial growth factor (VEGF) secretion may play a role in the pathogenesis of this tumor. We identified a patient with a very rare form of angiosarcoma arising from the retroperitoneum. Immunohistochemical analysis demonstrated widespread up-regulation of the hypoxic response pathway as a mechanism of enhanced VEGF expression. Disordered regulation of the hypoxic response pathway can result in the expression of factors such as VEGF and erythropoietin, which may promote autocrine tumor growth in angiosarcoma.     

Metastatic rhabdomyosarcoma of the thyroid gland, a case report

The thyroid gland is a known but an unusual site for metastatic tumors from various primary sites. Despite the fact that it is one of the largest vascular organs in the body, clinical and surgical cases have given an incidence of 3?% of secondary malignances of the organ. Nevertheless, thyroid metastases are not an exceptional finding at autopsy, they are encountered in 2?% to 24?% of the patients with malignant neoplasm. Soft tissue sarcomas metastatic to the thyroid are extremely rare as the majority of thyroid metastasis are caused by tumors of the kidneys, lungs, mammary glands, ovaries , and colon or by melanomas. We report a case of 22-years-old woman with right leg rhabdomyosarcoma metastatic to the thyroid gland.     

Experience of parathyroid cancer which required a differentiation from adenoma

A case was a 67-year-old man, who presented an inferior limb lassitude approximately in January 2009. We noted a thyroid gland mass by examination and referred for a further checkup. Biochemical examination of blood showed hypercalcemia, a high iPTH level of 2,190. The cervical echography evidence showed a mass image of the border regular in the back of inferior pole of left thyroid gland. It also revealed a mass image with the calcification in the inferior pole of left lobe of thyroid gland at the same time. We performed aspiration biopsy cytology for a thyroid gland mass, and it was class IIIa. We also performed parathyroidectomy and left lobe of thyroid gland ablative surgery because of a diagnosis of the hyperparathyroidism confirmed a primary parathyroid adenoma. Preoperative pathological diagnosis was adenoma. However, histopathology laboratory findings showed it to be of adenocarcinoma of the parathyroid, which metastasized into the thyroid. Many times parathyroid cancer preoperative diagnosis is inconclusive. So we are reporting our experience with parathyroid cancer metastases to the thyroid for reference.     

Tracheal carcinoma presenting as a thyroid tumor

Direct invasion of the trachea by an aggressive tumor originating in the thyroid gland is an unusual but well-documented phenomenon. The reverse situation, that is, a primary tracheal neoplasm invading by direct extension into the thyroid gland and presenting as a thyroid mass is apparently an even less common occurrence. A careful search of the literature has failed to reveal any recorded instances of this type of occurrence. We report a case of an adenoid cystic carcinoma arising in the trachea and invading the thyroid gland and recurrent laryngeal nerves causing vocal cord paralysis. The presentation of this lesion as a primary thyroid tumor is an unusual occurrence that serves to remind the clinician that in the differential diagnosis of thyroid masses one must include the possibilities of metastases or direct extension of a primary neoplasm from another organ.     

A Metastatic Ovarian Angiosarcoma Mimicking Hematologic Neoplasia at Diagnosis

Angiosarcomas are rare aggressive neoplasms of vascular endothelial origin with a high metastatic rate and poor prognosis. Involvement of the bone marrow by the angiosarcoma is exceedingly uncommon, and there have only been a few cases reported in the literature to date. Clinical manifestations and common laboratory findings of bone marrow involvement can mimic other more common bone marrow-replacing neoplasias such as lymphomas and acute leukemia. A definitive diagnosis is difficult to make from cytologic material, probably due to an associated bone marrow fibrosis, and requires bone marrow trephine biopsy with an immunohistochemical profile. Here we had the opportunity to study a case of metastatic angiosarcoma with positive cytologic findings and an unusual presentation that challenged its primary diagnosis.Key words: Angiosarcoma, Bone marrow metastasis, Splenomegaly, Bone marrow cytology     

Adenosquamous carcinoma originating from the thyroid gland--report of a case and review of the literature

Adenosquamous cell carcinoma originating from the thyroid gland is extremely rare. Only a few cases have been reported by light-microscopic observations. Like pure squamous cell carcinoma of the thyroid gland, it is generally thought to originate from the thyroid follicular epithelium and to be concerned with squamous metaplasia of the follicular epithelium. In our case, papillary adenocarcinoma was found in the thyroid gland, with the transition to squamous cell carcinoma. Immunohistochemical study for thyroglobulin and keratin further implied he transition of adenosquamous cell carcinoma in the thyroid gland. The origins of squamous cells in thyroid carcinoma are briefly discussed.     

Bilateral angiosarcoma of the breast detected by magnetic resonance imaging during pregnancy

Angiosarcoma of the breast is an aggressive malignancy of endothelial origin with a tendency for localregional recurrence. The involvement of angiosarcomas in the bilateral breasts has rarely been documented. Of note, due to its rarity and typically unclear clinical findings upon examination, the diagnosis of a contralateral lesion can be difficult, particularly in women during pregnancy. Here we present a rare case of bilateral angiosarcoma of the breast during pregnancy. A 32-year-old woman was referred to our unit with complaints of progressive swelling of the left breast, with tenderness. Magnetic resonance imaging showed a small, circumscribed high-intensity area in the contralateral breast, with pattern similar to that of the lesion found in the left breast. The contralateral lesion revealed only equivocal findings with the other diagnostic modalities. Diagnosed as angiosarcoma preoperatively, excision of the bilateral tumors was performed. Histological findings of the removed bilateral tumors were compatible with high-grade angiosarcoma of the breast.     

A case of the Stewart-Treves syndrome (post-mastectomy angiosarcoma) with special reference to a radiological study

Reported is the case of a 54-year-old female manifesting the Stewart-Treves syndrome (a postmastectomy angiosarcoma.) The patient had developed an angiosarcoma in her lymphoedematous right shoulder and lower neck develop ten years after a radical mastectomy for a papillo-tubular adenocarcinoma of the right mammary gland. Radiologically, a well-enhanced tumor was revealed by computed tomography, which angiographically had more stains and tumor vessels than usual for a mammary carcinoma and less than usually seen in a cavernous hemangioma. Histologically, this tumor showed less of a luminal differentiation than a conventional angiosarcoma and resembled a fibrosarcoma. Computed tomography and angiography were found useful in achieving a diagnosis and the subsequent therapy for this Stewart-Treves syndrome.     

Combination therapy for scalp angiosarcoma using bevacizumab and chemotherapy: a case report and review of literature

Bevacizumab, an angiogenesis inhibitor, is a recombined humanized monoclonal antibody against vascular endothelial growth factor and a promising therapeutic option for angiosarcoma management. This is a case report and review of the literature using bevacizumab and combination chemotherapy for angiosarcoma. The understanding of the effectiveness of combined therapy of bevacizumab and chemotherapy agents is still limited. The benefits of bevacizumab treatment for angiosarcoma will need to be weighed against the risks of venous thromboembolism in this population.Key Words: Angiosarcoma, chemotherapy, bevacizumab, angiogenesis inhibitor, combination therapy     

Angiosarcoma following MammoSite? partial breast irradiation

Angiosarcoma is a rare tumor of endothelial origin which commonly arises in small blood or lymphatic vessels. Its development in the treated breast following adjuvant external beam radiation is also rare. To our knowledge, there are no reported cases in the literature or MammoSite^? registry which describe the occurrence of angiosarcoma in the treated breast following MammoSite^? brachytherapy. This is a case report of a 74 year old female who developed angiosarcoma 4 years after receiving MammoSite^? balloon brachytherapy following surgical resection of a T1mic N0 M0 infiltrating ductal carcinoma.     

Angiosarcoma of the lung: 2 cases report and literature reviewed

Angiosarcoma is a kind of malignant tumors derived from vascular endothelial cell. Its incidence rate is very low. Pulmonary angiosarcomas are usually secondary tumors, and primary cases are less than 20 so far. Here we presented two cases of angiosarcoma involved the lung, and we only confirmed case 2 as a primary pulmonary angiosarcoma. The clinical characteristics, diagnosis, treatment options and prognosis of pulmonary angiosarcoma were reviewed in this article too.     

Extrapulmonary small cell: a novel case of small cell carcinoma of the thyroid gland

Neuroendocrine tumors comprise a large group of malignancies which share unique morphological features and are characterized by the presence of neuroendocrine markers such as synaptophysin, chromogranin-A, and CD56 (N-CAM), ranging from indolent tumors, such as carcinoid tumors, to aggressive tumors, such as small cell carcinoma. The lung is the most common site for primary neuroendocrine tumors. Extrapulmonary primary sites of small cell carcinoma are rare but have been documented arising from various sites including esophagus, stomach, colon and rectum, gallbladder, thymus, salivary gland, ovary, cervix, bladder, prostate, and skin. We present a case of small cell carcinoma arising from the thyroid gland, a site not previously described in the literature. A 59-year-old woman presented with a thyroid mass, which, after resection, showed small cell morphology and positive immunostains for TTF-1, synaptophysin, chromogranin-A, CD56, etc. Five months after diagnosis, she had widely metastatic disease. After a near-complete response to the first chemo-treatment, her disease progressed. Following local radiation and more rounds of chemotherapy, she succumbed to the disease, 15?months after diagnosis. Our patient had no pulmonary lesions at the time of diagnosis to suggest metastasis from the lung. Much like its pulmonary counterparts, this small cell carcinoma of primary thyroid origin displayed an aggressive clinical course and poor outcome. Although it shows early sensitivity to chemotherapy, small cell carcinoma remains a difficult-to-treat cancer with a poor prognosis and can rarely be seen originating in organs outside of the lung.     

Liposarcoma of the thyroid gland

The presumed first case of primary liposarcoma of the thyroid gland is presented, and the possible origin of this rare tumor from either mature fat cells or primitive mesenchymal cells is discussed.     

Complete radiographic response of primary pulmonary angiosarcomas following gemcitabine and taxotere

A small number of patients with angiosarcoma present each year, even fewer of whom have their primary origin site in the lungs. As such, specific treatments are not well defined for this tumor type. We report that the combination of gemcitabine and docetaxel may be an effective regimen for the treatment of angiosarcoma, as illustrated by the complete radiological response observed. In this case report, we review the clinical characteristics, prevalence and treatment options for angiosarcoma. In particular, we review the potential pitfalls and important attributes that should inform diagnosis.     

Efficacy of Tie2 Receptor Antagonism in Angiosarcoma

Angiosarcomas are malignant endothelial cell tumors with few effective systemic treatments. Despite a unique endothelial origin, molecular candidates for targeted therapeutic intervention have been elusive. In this study, we explored the tunica internal endothelial cell kinase 2 (Tie2) receptor as a potential therapeutic target in angiosarcoma. Human angiosarcomas from diverse sites were shown to be universally immunoreactive for Tie2. Tie2 and vascular endothelial growth factor receptor (VEGFR) antagonists inhibited SVR and MS1-VEGF angiosarcoma cell survival in vitro. In the high-grade SVR cell line, Tie2 and VEGF antagonists inhibited cell survival synergistically, whereas effects were largely additive in the low-grade MS1-VEGF cell line. Xenograft modeling using these cell lines closely recapitulated the human disease. In vivo, Tie2 and VEGFR inhibition resulted in significant angiosarcoma growth delay. The combination proved more effective than either agent alone. Tie2 inhibition seemed to elicit tumor growth delay through increased tumor cell apoptosis, whereas VEGFR inhibition reduced tumor growth by lowering tumor cell proliferation. These data identify Tie2 antagonism as a potential novel, targeted therapy for angiosarcomas and provide a foundation for further investigation of Tie2 inhibition, alone and in combinations, in the management of this disease.     

脾窦岸细胞血管瘤１例并文献复习

目的:报道1例少见的脾脏窦岸细胞血管瘤(littoral cell angioma,LCA),以提高临床医师对该疾病的认识。方法:对1例LCA的临床、病理特征、免疫组化及术后随访情况进行分析并复习文献。结果:LCA具有内皮标记如CD31以及组织细胞标记如CD68双重分化的特征。结论:LCA是脾脏的良性肿瘤,病理上易与窦岸细胞血管内皮瘤和窦岸细胞血管肉瘤等混淆,诊断依靠病理组织学表现及免疫组化特点。行脾切除术可治愈,预后良好。     

Primary T-cell lymphoma of the thyroid: case report and review of the literature

Here we report a rare case of primary T-cell lymphoma of the thyroid gland. A 32-year-old Chinese man was admitted to our hospital because of marked goiter and subcutaneous nodules. Computed tomography (CT) scan of the neck disclosed diffuse enlargement of the thyroid gland. A diagnosis of peripheral T-cell lymphoma was made according to cytopathological findings of fine-needle aspiration (FNA) of the thyroid gland and skin biopsy. Immunohistochemistry stain showed that the tumor was positive for CD3 and negative for CD20. Clonal T-cell receptor (TCR-γ) gene rearrangements were demonstrated by polymerase chain reaction. After six cycles of combination chemotherapy (bleomycin, cyclophosphamide, doxorubicin, vincristine, and prednisone), the thyroid retracted to normal size gradually.     

Angiosarcome du sein : à propos de deux cas

Angiosarcoma is a rare type of breast cancer; it has the worst prognosis of all breast malignancies and it is difficult to diagnose. Its definition is pathological; it is a primary malignant proliferation of endothelial cells of vascular tissue of the mammary gland. We report two cases of breast angiosarcoma observed at the Maternity of Oranges and at the National Institute of Oncology. The preoperative diagnosis was raised on the clinical and pathological data. The treatment was essentially surgical. The evolution was marked by complete remission in both cases. In light of these two observations and the literature review, we tried to update on the various aspects of this disease.     

Postirradiation angiosarcoma of the breast--a case report

Angiosarcoma of the breast is an uncommon neoplasm but coincident angiosarcoma with infiltrating ductal carcinoma is not yet reported. This paper describes a case of angiosarcoma which developed after 4 years of a wide excision and adjuvant radiotherapy for a ductal carcinoma of the breast. Due to latent period of only 4 years radiotherapy cannot be blamed as an aetiological factor in this case but neither is it possible to exclude it as a causative factor.