A case of microangiopathic hemolytic anemia associated with breast cancer: Improvement with chemoendocrine therapy

Microangiopathic hemolytic anemia (MAHA) is a term which describes the association of hemolytic anemia with red cell fragmentation caused by microangiopathy mechanically. This paper reports a 45-year-old woman with bone metastases from breast cancer. She developed MAHA and disseminated intravascular coagulation (DIC). Although the prognosis of MAHA associated with malignant tumor has been very poor, she achieved remission of the syndrome after chemoendocrine therapy.     

Malignant (fibrous) histiocytoma of bone--fact or fancy?.

Malignant (fibrous) histiocytoma is currently defined as a malignant primary bone tumor that contains a mixture of fibrogenic cells and cells that are histologically similar but which appear to be histiocytic. In this type of histiocytoma the nuclei are often indented; cytoplasm is usually abundant and may be slightly foamy; nucleoli are often large; and multinucleated malignant cells are usually a prominent feature. Many dedifferentiated chondrosarcomas, osteosarcomas, and fibrosarcomas of bone contain areas that resemble what we regard as malignant (fibrous) histiocytoma. When the entirety of a malignant tumor of bone fits the outlined histologic pattern, the designation of malignant (fibrous) histiocytoma seems appropriate. From 158 fibrosarcomas of bone and 962 osteosarcomas of bone in our files, 35 tumors were segregated because they appeared to be properly designated as malignant (fibrous) histiocytoma. A wide age range was represented by affected patients, and a large variety of bones harbored these tumors. Approximately one-third of patients eligible for 5-year follow-up were long-term, symptom-free survivors. Four deaths occurred from the tumor after more than 5 years, and one patient had radiographic evidence of pulmonary metastasis 7 years after amputation. Radiation therapy has been curative in at least two cases. The correct designation for these tumors in the light of current knowledge is malignant tumor, consistent with malignant (fibrous) histiocytoma.     

Two cases of stromal sarcoma, so-called malignant fibrous histiocytoma of breast treated with reduction surgery

CASE 1: A 64-year-old woman with right breast cancer had a partial mastectomy and radiotherapy four years ago was admitted to our hospital because of right breast mass. The vacuum associated biopsy of tumor resulted in breast sarcoma, thus a nipple-spearing mastectomy was performed. The final histological diagnosis was stromal sarcoma, and was identical to histological findings of malignant fibrous histiocytoma. Three months after the operation, a chest wall recurrence appeared. Although tumor resection with latissimus dorsi flap was performed, there were pleural dissemination and malignant pleural effusion. She died six months after the first surgery. CASE 2: A 60-year-old woman was admitted to our hospital because of left breast mass, but she refused a further clinical examination. She readmitted fourteen months later due to a huge sized left breast mass with necrosis and smelling discharge. CT scan showed a huge tumor of left breast and multiple lung metastases. Biopsy of the tumor resulted in breast sarcoma. Total mastectomy with split thickness skin grafting was performed. The final histological diagnosis was identical to the first case. A local recurrence appeared thirty days after the operation, and she died fifty-one days after the operation.     

An autopsy report of malignant fibrous histiocytoma of the left atrium and a review of the pertinent literature

A 30-year-old male complaining of fever was admitted to hospital and a diagnosis of a malignant, fibrous histiocytoma (MFH) was established after a biopsy examination. Antitumor chemotherapy and Co60 irradiation was initiated. The patient, however, suddenly died of cardiac arrest 9 months after admission. The autopsy revealed a polypoid, yellow-white tumor (5 cm in diameter) arising from the antero-lateral wall of the left atrium and occupying the chamber. Twenty-two cases of MFH (21 previous reports and 1 current case) arising from the heart are reviewed.     

Malignant fibrous histiocytoma of the scrotal wall--a case report

The case involves a 64-year-old male who was found to have a gradually enlarging tumor which was responsible for a slight tenderness in his scrotum for a month. Tumor excision was performed, and the tumor size was found to be 3 X 2 X 2 cm. A histologic examination of this tumor revealed a malignant fibrous histiocytoma of the scrotal wall. The patient received UFT chemotherapy. Five months after the excision, the patient was rehospitalized with a local recurrence in the scrotum. After a second operation, the patient was treated with radiotherapy.     

Acute Disseminated Intravascular Coagulation in Neuroendocrine Carcinoma

Malignancy is a common cause of disseminated intravascular coagulation and usually presents as a chronic disorder in solid organ tumours. We present a rare case of recurrent acute disseminated intravascular coagulation in neuroendocrine carcinoma after manipulation, firstly, by core biopsy and, later, by cytotoxic therapy causing a release of procoagulants and cytokines from lysed tumour cells. This is reminiscent of tumour lysis syndrome where massive quantities of intracellular electrolytes and nucleic acid are released, causing acute metabolic imbalance and renal failure. This case highlights the potential complication of acute disseminated intravascular coagulation after trauma to malignant cells.Key words: Disseminated intravascular coagulation, Neuroendocrine carcinoma, Tumour lysis syndrome     

32例初发初治晚期恶性肿瘤合并血小板减少临床分析

目的:探讨初发初治晚期恶性实体瘤合并血小板减少的常见病因及其对化疗的影响.方法:对32例初发初治晚期恶性肿瘤合并血小板减少的病因及化疗后的结果进行回顾性分析.结果:32例引起初发初治晚期恶性实体瘤合并血小板减少的病因:脾功能亢进6例,弥散性血管内凝血5例,骨髓增生减低2例,巨核系成熟障碍3例,免疫性因素14例,肿瘤侵犯骨髓2例;其中30例进行了化疗,PR19例,SD 11例.结论:初发初治晚期恶性实体瘤合并血小板减少,大多数可耐受化疗,并从化疗中获益.     

Cardiac metastases in malignant fibrous histiocytoma. A case report

Malignant fibrous histiocytoma metastasizing to the left ventricle is an uncommon form of cardiac malignancy. This report describes a rare case of left ventricular metastases from a malignant fibrous histiocytoma of the posterior compartment of the right thigh, recurring five years after treatment with surgery, hyperthermic perfusion of the limb and radiotherapy. As the patient presented symptoms of cardiac tamponade, open heart surgery was performed through a median sternotomy; however, the tumor was not resectable and only a biopsy was performed. A partial response was obtained with standard and high-dose chemotherapy with peripheral blood progenitor cell transplantation. The response continued to improve with immunotherapy. The patient returned to normal physical activity. He died four years later due to a ventricular arrhythmia.     

Postirradiation malignant fibrous histiocytoma in a patient with squamous carcinoma of the uterine cervix--an autopsy case

An autopsy case of postirradiation sarcoma is described. The tumor, a malignant fibrous histiocytoma, occurred in the radiation fied 7 years after postoperative external beam radiation therapy (6000 rad) for squamous cell carcinoma of the cervix. To our knowledge, 11 cases of postirradiation malignant fibrous histiocytoma have been reported in the literature and only one occurrence of this neoplasm after treatment for cervical cancer has been described. We review the literature on postirradiation sarcoma with special attention to malignant fibrous histiocytoma.     

Radiation-induced intracranial malignant fibrous histiocytoma.

An autopsy case of radiation-induced intracranial malignant fibrous histiocytoma (fibroxanthosarcoma) is reported. The tumor developed in the region of the sella turcica 11 years after high dose radiotherapy of a chromophobe adenoma of the pituitary. The tumor had infiltrated the base of the brain as well as the base of the skull. Metastases were not found. The tumor was composed of an admixture of bizarre fibroblasts, histiocytes and giant cells, xanthoma cells and siderophages, with a storiform fibrous stroma. This appears to be the first documented instance of a malignant fibrous histiocytoma occurring intracranially after local x-irradiation.     

Primary malignant fibrous histiocytoma of spleen with spontaneous rupture: a case report and literature review

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma that mainly occurs in the lower and upper extremities, trunk and retroperitoneum. However, primary MFH of the spleen is especially rare. Only 11 cases of splenic MFH have been reported in the English literature. In this report, we describe a 35-year-old man who was found to have a large accumulation of free fluid in the abdominal cavity and a tumor mass 6 cm in diameter with rupture within the spleen by the abdominal ultrasonography. A splenectomy was performed and the histological diagnosis was malignant fibrous histiocytoma. The patient died 7 months after the operation as a result of generalized metastasis. Compared with the 11 previously documented patients of splenic MFH, our patient is the youngest and the first case with spontaneous rupture, which makes our case exceedingly rare. A literature review of primary MFH of spleen is also provided.     

Malignant fibrous histiocytoma in the craniocervical junction presenting with severe occipitalgia

We report a patient who complained of severe occipitalgia caused by destruction of the atlantooccipital joint by tumor invasion. Her symptoms were relieved by tumor resection and occipitocervical fixation. Histological examination of the resected tumor revealed that the tumor cells had an irregular arrangement, remarkable atypia, and pleomorphism with multinucleated bizarre giant cells. The tumor demonstrated no definitive sarcoma differentiation and was identified as malignant fibrous histiocytoma. After tumor resection, the patient received adjuvant radiation and chemotherapy. The tumor regrew outside the radiation field. Chemotherapy with ifosfamide, cisplatin, and etoposide caused remarkable tumor reduction, but suspension of chemotherapy resulted in tumor recurrence. The results of our drug protocol suggest that this regimen is feasible as postoperative adjuvant chemotherapy for malignant fibrous histiocytoma. The role of adjuvant chemotherapy and radiation therapy for this highly malignant rare tumor should be evaluated in a prospective study with precise histological diagnosis.     

Malignant fibrous histiocytoma of the heart. A case report and review of the literature

A case report of a 28-year-old woman with malignant fibrous histiocytoma (MFH) of the left atrium is presented, and the six previous reports of this rare cardiac tumor are reviewed. A tendency for malignant fibrous histiocytoma of the heart to occur in the left atrium of young women is suggested; this sarcoma's usual location is in the soft tissue of elderly men. The apparent predilection for the left atrium is unique among cardiac malignancies. Careful pathologic study is necessary to differentiate the uniformly fatal MFH of the heart from the more common benign atrial myxoma.     

Retroperitoneal malignant fibrous histiocytoma--report of a case

A 52-year-old man with a three-month history of left epigastralgia and body weight loss was referred to us for a possible abdominal tumor in March 1984. Retroperitoneal malignant tumor was suspected by ERCP, angiography, US, and CT. At laparotomy, a child's-head-sized retroperitoneal tumor, which weighed 1,800 g, was resected. Pathological examination revealed the diagnosis of retroperitoneal malignant fibrous histiocytoma (MFH). He died because of recurrence in the retroperitoneum six months after the operation. We selected 26 Japanese cases of retroperitoneal MFH and discussed their clinical findings and prognosis.     

Lymphocytosis in a patient with malignant fibrous histiocytoma

Malignant fibrous histiocytoma is an uncommon soft-tissue sarcoma. Patients with malignant fibrous histiocytoma may present with unusual clinical findings and an apparent association with malignant hematopoietic disease has been reported. We describe a patient in whom a diagnosis of chronic lymphocytic leukemia was made simultaneously with discovery of a large, retroperitoneal malignant fibrous histiocytoma. Following surgical debulking of the tumor, the patient's blood and bone marrow findings returned to normal.     

腹膜后巨大恶性纤维组织细胞瘤误诊1例并文献复习

目的观察腹膜后恶性纤维组织细胞瘤的临床表现、病理形态及免疫组化特点，吸取误诊教训。方法通过对1例腹膜后恶性纤维组织细胞瘤的临床表现、光镜形态及多次免疫组化结果进行观察，明确诊断，并复习相关文献。结果恶性纤维组织细胞瘤多发于中老年男性。根据镜下所见及第一次免疫组化结果曾诊断为巨大恶性神经鞘膜瘤。后又经多次免疫组化结果S-100（-），NF（-），排除恶性神经鞘膜瘤的可能；经多次专家会诊及远程会诊，确诊为腹膜后巨大恶性纤维组织细胞瘤。结论原发性腹膜后恶性肿瘤比较多样化，因此他的确诊依赖于镜下形态和可靠的免疫组化结果，这些经验教训值得大家借鉴。     

Malignant transformation of oligoastrocytoma: a case report

We report a case of oligoastrocytoma resembling dysembryoplastic neuroepithelial tumor (DNT) with malignant transformation. A 35-year-old woman presented with headache and generalized convulsion in May 2003. Magnetic resonance imaging (MRI) revealed an extensive left temporal lobe tumor. She underwent partial resection of the tumor under awake surgery, while preserving her language function. The surgical specimen showed that the majority of the tumor was composed of a glioneuronal element. However, there was also an abundant oligoastrocytoma component. Therefore, our first pathological diagnosis was oligoastrocytoma and DNT. She then underwent radiation therapy. The tumor recurred at the left temporal lobe in June 2005. She then underwent open biopsy. The pathological diagnosis was anaplastic oligoastrocytoma with a MIB-1 staining index of 79%. She received PAV (procarvazine, ACNU, and vincristine) chemotherapy, and the tumor subsided transiently. However, she died 3 years after the first operation. Although the histological findings of the first surgical specimen closely resembled those of DNT, radiologic findings and clinical course were different from those of DNT. The authors concluded that this tumor could be a malignant transformation of oligoastrocytoma mimicking DNT, and we wish to give warning that the presence of a glioneuronal component is not an absolute benign hallmark.     

32例初发初治晚期恶性肿瘤合并血小板减少临床分析

目的：探讨初发初治晚期恶性实体瘤合并血小板减少的常见病因及其对化疗的影响。方法：对32例初发初治晚期恶性肿瘤合并血小板减少的病因及化疗后的结果进行回顾性分析。结果：32例引起初发初治晚期恶性实体瘤合并血小板减少的病因：脾功能亢进6例,弥散性血管内凝血5例,骨髓增生减低2例,巨核系成熟障碍3例,免疫性因素14例,肿瘤侵犯骨髓2例;其中30例进行了化疗,PR19例,SD 11例。结论：初发初治晚期恶性实体瘤合并血小板减少,大多数可耐受化疗,并从化疗中获益。     

Benign and malignant fibrous histiocytomas of the soft tissues: functional characterization of the cultured cells

Cell cultures were carried out from fresh tumor tissues obtained from seven cases of histiocytic tumors of the soft tissues including four of malignant fibrous histiocytoma, two of benign fibrous histiocytoma, and one of giant cell tumor of tendon sheath. The cultured cells were studied by light and electron microscopy, rosette formation for surface receptors, immune phagocytosis, and enzyme cytochemistry. The culture of each tumor revealed a mixture of histiocyte-like cells, fibroblast-like cells, amd intermediate forms. Bizarre giant cells were numerous in the early cultures of malignant fibrous histiocytoma, but they were absent in the cultures of benign fibrous histiocytoma and giant cell tumor of tendon sheath. In all the seven cases the cultured cells exhibited several features characteristic of histiocytes: (1) surface receptors for the Fc-portion of immunoglobulin G; (2) surface receptors for the third component of complement; (3) immune phagocytosis of opsonized erythrocytes; and (4) lysosomal enzymes including alpha-naphthyl butyrate esterase, and acid phosphatase. These findings suggest that the histiocytic tumors of the soft tissues in discussion actually comprise the cells that have the functional property of histiocyte. The tumor cells in the surgical specimens presented essentially the same ultrastructural and enzyme histochemical characteristics as those of the cultured cells.