Radiation-induced breast sarcoma

BACKGROUND: The purpose of this study was to examine the presentation, treatment, and prognosis of patients with radiation-induced sarcomas after adjuvant radiotherapy for breast cancer. METHODS: This was a retrospective review from 1975 to 2001 of patients who presented with a sarcoma in an irradiated field after surgical treatment for breast cancer. RESULTS: Thirty-four women were included. Six had undergone breast-conserving therapy and 28 had mastectomy for primary breast carcinoma. All patients received postoperative radiation. The mean time to diagnosis of sarcoma was 152 months (range 40 to 372). Twenty-three of 34 patients (68%) had recurrence of the sarcoma after resection, and 22 patients (65%) died of their disease. Patients with no evidence of disease at follow-up had a mean tumor size of 4.2 cm +/- 0.7 (n = 6), compared with 8.1 cm +/- 1.2 (n = 10) for patients who died of their disease (P = 0.030). CONCLUSIONS: Radiation-induced sarcoma is a late complication of definitive treatment for breast carcinoma. The prognosis of such patients is poor, with two thirds dying of their disease. In the present series, improved survival was associated with smaller tumor size at presentation.     

Renal primary angiosarcoma

Angiosarcomas account for 2% of all soft tissue sarcomas and of them, primary renal angiosarcomas represent 1%. Twenty-four cases have been published in the English specialised literature. We report the second case to be described in a middle-aged female, with pulmonary metastases at diagnosis, and fatal outcome despite surgery and chemotherapy.     

Orthotopic liver transplantation for epithelioid haemangioendothelioma.

AIMS: To report seven cases of epithelioid haemangioendothelioma (EHE) of the liver, a rare, low-grade malignant neoplasm of vascular origin that have been treated in our institution. MATERIALS AND METHODS: Patients with ages ranging from 25 to 60 years presenting mainly with non-specific symptoms, such as right upper quadrant abdominal pain or weight loss. The tumours presented as multiple, nodular lesions involving both lobes of the liver. This type of tumour is often difficult to diagnose, with the final diagnosis being established only by histological examination. The key to diagnosis was the demonstration of cells containing factor VIII-related antigen. RESULTS: Five patients underwent orthotopic liver transplantation (OLT), four of whom are alive at a median follow up 38 months (11-88 months). One patient died of recurrent of disease at 88 months. Two patients did not receive an OLT since they presented with extrahepatic metastatic disease; they died at 21 and 25 months from diagnosis. CONCLUSION: Orthotopic liver transplantation may be considered as a potentially curative treatment for this rare form of tumour when the disease is confined to the liver.     

肝原发性血管肉瘤（附三例报告）

本文报告了３例肝原发性血管瘤，男性２例，女性１例，平均年龄３２．７岁，占同期肝脏恶性肿瘤检出率的０．６８％（３／４３６），临床表现为上腹痛及包块，血清ＨＢｓＡｇ及ＡＦＰ阴性，本组资料还进行了网状纤维染色及免疫组织化学标记，本文讨论肝血管肉瘤的临床病理特点，诊断及鉴别诊断。     

Primary spindle-cell sarcomas of the breast: diagnosis by fine-needle aspiration

Primary breast sarcomas (excluding cytosarcoma phyllodes and its sarcomatous recurrences) are rare neoplasms. Few have been described in the aspiration cytology literature. We report the cytologic features of two cases of stromal sarcoma (both with the pattern of malignant fibrous histiocytoma) and two cases of angiosarcoma. The dominant cytologic features included individual atypical spindle cells and fragments of collagenous stroma. Tumor giant cells were present in one stromal sarcoma. Features of possible significance in the diagnosis of angiosarcoma include obvious vessel formation by atypical spindle cells, bridging of adjacent tumor fragments by spindle cells, and microacinar structures lined by atypical spindle cells. The differential diagnostic considerations in spindle-cell breast aspirations are discussed.     

Angiosarcoma associated with radiation therapy after treatment of breast cancer. Retrospective study on ten years

PurposeThe breast sarcoma induced by radiation therapy is rare but increasing, given the increased long-term survival of patients receiving radiation therapy. Fibrosarcoma, histiocytofibroma and angiosarcoma are the most common breast sarcoma. Angiosarcoma is the most common after breast cancer treated by radiation therapy, often diagnosed too late, with a severe prognosis and a high rate of recurrence. However, because of the low incidence of angiosarcoma associated with radiation therapy (AAR), the benefit of radiation therapy in breast cancer treatment outweighs the risk to develop angiosarcoma. The aim of this study is to evaluate these rare cases of AAR diagnosed in eastern Belgium in comparison to the data from the literature.Patients and methodsNine cases of AAR after radiation for breast ductal carcinoma were included in this retrospective study. AAR was diagnosed according to Cahan criteria between January 2007 and December 2016. Latency, incidence, management and prognosis are comparable to the literature.Results, conclusionThe median latency was 10 (4–24) years, the incidence of AAR in the East Belgian area was 0.09% of the patients irradiated on the same period. Patients were treated by surgery with wide local excision with or without reconstructive surgery, without radiotherapy and chemotherapy treatment. Kaplan-Meier analysis showed median overall survival of 61.8 months, patient survival of 55.6% at one year and 29.6% at five years. With the constant progress of medicine and its technologies, it would be possible to limit the occurrence of AAR or to diagnose it at an earlier stage.     

Epithelioid angiosarcoma arising in a deep-seated plexiform schwannoma: a case report and literature review

Angiosarcoma developing within a schwannoma is rare, and only 8 cases have been reported in the literature. Its association with a plexiform schwannoma has never been reported. We describe an epithelioid angiosarcoma arising in a plexiform schwannoma of the sciatic nerve in a 73-year-old man not known to have von Recklinghausen disease. Grossly, the tumor forms a long tubular mass with a multinodular growth pattern on the cut surface. Microscopically, these nodules display characteristics of a schwannoma, including Antoni A, Antoni B, and Verocay bodies. Adjacent to these nodules are high-grade anaplastic epithelioid cells growing in solid sheets, cords, and nests with focal vasoformative channels. The anaplastic cells are immunoreactive to CD31, CD34, and factor VIII related antigen, supporting their endothelial differentiation. Local recurrence and multiple pulmonary metastasis occurred 3 months after complete excision of the tumor. This case demonstrates the first occurrence of an epithelioid angiosarcoma in a deep-seated plexiform schwannoma with an aggressive clinical behavior.     

Soft tissue tumors of the urinary bladder Part II: malignant neoplasms

Most bladder tumors arise from the urothelium. However, there are several uncommon but significant malignant bladder lesions that must be differentiated from urothelial carcinomas and from benign lesions of the bladder. The second half of this two-part review will describe rare nonurothelial malignant tumors of the urinary bladder including leiomyosarcoma, rhabdomyosarcoma, angiosarcoma, malignant fibrous histiocytoma (undifferentiated sarcoma), primitive neuroectodermal tumor, malignant peripheral nerve sheath tumor, hemangiopericytoma, and alveolar soft-parts sarcoma. Common clinical presentations, morphologic characteristics, and immunohistochemical features are described to aid the practicing pathologist in the identification of these entities. Because the distinction between malignant and benign lesions has significant therapeutic and prognostic implications, key factors for differentiating them are presented.     

Hepatic angiosarcoma manifested as recurrent hemoperitoneum

Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum.     

A case of mixed adult Wilms’ tumour and angiosarcoma responsive to carboplatin, etoposide and vincristine (CEO)

Here we report an unusual case of mixed Wilms’ tumour and angiosarcoma in a 38-year-old female patient who presented with haematuria and right lower back pain. A computed tomographic (CT) scan confirmed a massive renal tumour associated with extensive retroperitoneal lymph node involvement, bony metastases and a right hip fracture. She was initially managed with palliative nephrectomy, which was followed by rapid postoperative deterioration. Histopathology revealed differentiated adult Wilms’ tumour with renal angiosarcoma, whereas the pathology of the para-aortic lymph node and bone metastasis revealed angiosarcoma only. In view of her cachexia and cytopaenia, emergency chemotherapy was initiated using a modified regimen of carboplatin, etoposide and vincristine (CEO) in preference to the more traditional but less well-tolerated VAC (vincristine, actinomycin D, cyclophosphamide). Four cycles of this protocol yielded a dramatic response on re-staging CT scan. This case suggests that highly angiogenic tumours such as angiosarcoma may be effectively palliated using agents usually reserved for refractory Wilms’ tumour, and supports the view that adult Wilms’ tumour is more sensitive to such agents.