A case of interleukin-6-producing malignant fibrous histiocytoma originating in the heart.

Primary cardiac malignant fibrous histiocytoma is extremely rare and its pathophysiological characteristics remain largely unknown. We treated a female patient with persistent fever and disseminated intravascular coagulation. Since ultrasonic echocardiography revealed the presence of a cardiac tumor and her serum interleukin-6 level was elevated, we speculated she had a cardiac myxoma. Histological examination of the surgically resected specimen, however, revealed that the tumor was malignant fibrous histiocytoma. Although her disseminated intravascular coagulation and heart failure were transiently improved after operation, local recurrence and systemic metastasis occurred and she died 7 weeks after operation. Using the autopsied specimen, we examined whether the malignant fibrous histiocytoma constitutively synthesized interleukin-6. The interleukin-6 content in the tumor was high, consistent with interleukin-6 production by the tumor. This was confirmed by immunohistochemical analysis. To our knowledge, this is the first report demonstrating interleukin-6 production by a cardiac malignant fibrous histiocytoma.     

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.     

Management of cardiac metastasis from malignant fibrous histiocytoma

The management of a case of malignant fibrous histiocytoma with cardiac metastasis is described in this article. Because of the extent of disease in the right ventricle, surgical resection was impossible. After disease progression on chemotherapy, a right atrial to pulmonary artery shunt was performed. Although the surgical procedure was successful, the patient died from progressive disease. Management of cardiac metastasis is also discussed.     

Histiocitoma fibroso maligno de pared abdominal

Malignant fibrous histiocytoma is a type of soft tissue sarcoma. Although is usually described as a primary neoplasm it has also been related to previous radiation therapy. We report the case of a 49-year-old woman with a history of chemo-radiotherapy five years before due to a cervix cancer. Five years after, a malignant fibrous histiocytoma was discovered in tattoo radiation fields. A wide resection of the tumor was performed and the abdominal wall defect was covered. Diagnosis of this type of tumors remain controversial, they are very aggressive and surgery is still the treatment of choice.

     

36例恶性纤维组织细胞瘤的术后放疗临床疗效分析

目的探讨恶性纤维组织细胞瘤手术切除 放射治疗的临床疗效。方法对36例恶性纤维组织细胞瘤患者行手术切除 放射治疗。结果5年单纯局部复发率27.78%(10/36);单纯远处转移率13.89%(5/36);局部复发合并远处转移8.33%(3/36);1、3、5年生存率分别为97.22%(35/36)、75.00%(27/36)、63.89%(23/36)。结论恶性纤维组织细胞瘤手术切除 放射治疗的疗效较好,值得进一步深入研究。     

Echocardiography Diagnosis of Cardiac Tumours –Royal Children's Hospital,Melbourne Experience, 1979–1983.

Six cardiac tumours have been detected over the preceding five year period and are reviewed. There were four left ventricular rhabdomyomas, one right ventricular myxoma and one intra-pericardial teratoma with a malignant yolk sac component. One patient known to have tuberous sclerosis died at home and at autopsy was discovered to have multiple left and right ventricular rhabdomyomas. The second infant had the diagnosis of a broad-based left ventricular rhabdomyoma made on the basis of the left ventricular angiogram, has mild to moderate left ventricular outflow tract obstruction and has been treated conservatively for three and a half years. Two remaining infants with left ventricular rhabdomyomas had severe left ventricular outflow tract obstruction and underwent emergency excision. The infant with the right ventricular myxoma had severe right ventricular outflow tract obstruction. The infant with the intra-pericardial teratoma was moribund with cardiac compression. These two also underwent emergency excision. All four surgical cases are alive and well six months to five years after surgery. Echocardiography has played an increasing. important role both in the diagnosis and subsequent follow-up of these patients.     

Malignant fibrous histiocytoma (MFH) arising within a cholecystectomy incision: a cause-and-effect relationship is possible between previous surgery and the development of a malignant fibrous histiocytoma within the operative site

We present a report of a patient who, after an uneventful cholecystectomy, developed an incisional mass that proved to be a malignant fibrous histiocytoma and had to be excised. Five months later, the patient developed a similar incisional mass, which was shown to be a malignant fibrous histiocytoma and again had to be excised. The rarity of such a case and the details of the patient's case history are reported. The implications of a possible relationship between previous surgery, the patient's healing response, and the development of these tumors are discussed.     

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 Presenting as an Intraventricular Mass Five Years after Incidental Detection of a Mass Lesion

Malignant fibrous histiocytoma is a rare intracranial neoplasm. It usually presents as a meningeal mass but occurs also intraaxially. Few information is available on cellular origin, premalignant histologic stages and time course of malignant transformation. We report a case of a primary intraventricular malignant fibrous histiocytoma in a patient who five years prior to clinical manifestation of the malignancy was found to have an intraventricular mass with benign CT characteristics.     

Malignant fibrous histiocytoma of the heart presenting as an atrial myxoma.

A 37-year-old woman presented with signs and symptoms suggestive of mitral stenosis and insufficiency. Subsequent evaluation demonstrated a left atrial tumor which was suspected clinically and at the time of initial surgery to be an atrial myxoma. Histologic examination revealed that the tumor was actually a malignant fibrous histiocytoma. Despite near total removal at surgery and subsequent radiotherapy, the tumor recurred within six months in the left atrium. Re-excision was followed by a third recurrence in the same site.     

Localized fibrous malignant peritoneal mesothelioma in an operated patient with massive hemorrhage to the peritoneal cavity

We reported an operated patient with localized peritoneal mesothelioma. The patient was a 39-year-old man who had occasionally complained of left hypochondralgia for 5 years before admission. Progressive anemia due to hemorrhage into the peritoneal cavity was noted after admission. Peritoneoscopy revealed the bleeding to be located between the left lobe of the liver and the anterior wall of the stomach. On the 22nd hospital day, surgery was performed and a fist-sized tumor was resected. Microscopically, it was fibrous malignant type of the peritoneal mesothelioma. Chemotherapy was performed 3 times after the operation, and the patient is living and well 1 year and 8 months postoperatively.     

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.     

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.     

Osteosarcoma: intra-arterial treatment of the primary tumor with cis-diammine-dichloroplatinum II (CDP). Angiographic, pathologic, and pharmacologic studies

Intra-arterial CDP was utilized to treat the primary tumor in 11 pediatric patients with osteosarcoma and in one with malignant fibrous histiocytoma. The investigation commenced with a phase I-II pilot study in four osteosarcoma patients. A dose of 150 mg/m2 was found to be safe and effective in producing a clinical response. This was followed by a definitive study in the remaining seven osteosarcoma patients and in the one malignant fibrous histiocytoma patient. The results were assessed by specific clinical, pharmacologic, radiographic and pathologic criteria. The overall response in the definitive study was 50% with two patients exhibiting total tumor destruction. The success of intra-arterial CDP was attributed to its ability to achieve high local drug concentration and tumor penetration. This was demonstrated by pharmacologic studies.     

Malignant fibrous histiocytoma of bone. A review of 13 cases and an ultrastructural study

Observations on 13 patients with primary malignant fibrous histiocytoma of bone were reported. Included were nine male and four female patients, ranging in age from 6 to 81 years (mean, 44.8 years). Pain was the most common complaint. The interval from the first symptom to the initial treatment varied from 2 months to 20 years. Seven tumors arose in the knee region. Roentgenologically, most of the lesions presented with an osteolytic and destructive appearance. Histologically, highly variable morphologic features existed. The storiform-pleomorphic pattern was found in every tumor, although it was not necessarily pathognomonic for malignant fibrous histiocytoma of bone. Surgery, radical or incomplete, was the primary treatment for all but one patient. Lymph node metastasis was present in three. Five patients died of the disease from 3 to 79 months after the diagnosis (mean, 28 months), all exhibiting metastasis to the lung. Gaucher's body-like structure demonstrated in the electron microscopic study shows the histiocytic quality of malignant fibrous histiocytoma of bone.     

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.     

A Japanese patient with Li-Fraumeni syndrome who had nine primary malignancies associated with a germline mutation of the p53 tumor-suppressor gene

We describe a patient who had nine primary malignant tumors and a germline mutation in the p53 tumor-suppressor gene, characteristically found in the Li-Fraumeni syndrome (LFS). A 15-year-old girl with no family history of cancer was referred to our hospital because of pain and swelling of the right knee. Osteosarcoma was diagnosed. The patient received chemotherapy followed by surgery and had a remission. After the age of 28 years, nine primary malignant tumors developed successively, including right breast cancer, colon cancer, malignant fibrous histiocytoma (MFH) of the abdominal wall, right lung double cancers, bilateral breast cancers, and MFH of the left thigh. This is the second highest number of types of primary malignant tumors to be reported in LFS. All tumors were treated by a multidisciplinary approach, including surgery. Genetic analysis revealed a germline missense mutation in the p53 gene (c.659 A > G), resulting in Y220C, which has been reported in three families with LFS. The patient died of lung metastasis from MFH at the age of 37 years. Despite the multiple tumors, repeated induction of remissions resulted in long survival. Our findings suggest that a multidisciplinary approach to treatment, including surgery, is beneficial in patients with LFS.     

Malignant fibrous histiocytoma of the scrotum

A 72-year-old man was hospitalized for a gradually enlarging left scrotal mass. Physical examination revealed a 3 X 4 cm. rock hard non-tender mass fixed to adacent tissue. The tumor invaded the skin of scrotum and showed ulcer formation. Surgical therapy was performed under the diagnosis of left scrotic tumor. The resected mass was examined histologically, and the pathologic diagnosis was of malignant fibrous histiocytoma of the left scrotum was made.     

Metastatic intracerebral malignant fibrous histiocytoma from the lung

Malignant fibrous histiocytoma usually develops in the extremities, peritoneal or retroperitoneal space and the thigh. The occurrence in the lung and brain is quite rare. We report two cases of metastatic intracerebral malignant fibrous histiocytoma from the lung. The first patient developed cerebral metastasis six years after treatment of the primary site, and the second one died two months after the diagnosis of generalized metastases. Both were quite resistant to radiation therapy.