腹部恶性纤维组织细胞瘤47例临床和预后分析

目的探讨腹部恶性纤维组织细胞瘤(MHF)的临床病理特点、治疗策略及预后相关因素。方法回顾性分析1970年1月至2000年1月间天津医科大学附属肿瘤医院47例腹部恶性纤维组织细胞瘤患者手术切除治疗情况。所有患者均经手术病理证实,病变单一。结果术后随访中共有32例患者出现复发、转移,术后单纯局部复发13例(29.5%),出现肺及胸膜转移7例(15.9%),肝转移4例(9.0%),骨转移6例(13.6%),肾脏转移2例(4.5%),复发 转移5例(11.4%)。术后1,3,5年无瘤生存率分别为65.9%、40.9%和29.5%,累计生存率分别为70.5%、52.3%和31.8%,中位生存期为18个月。影响预后的主要因素为肿瘤发生的部位、治疗方式、病理类型、肿瘤的局部复发等。以手术为主的综合治疗较单纯手术及放化疗预后好,术后辅以放疗可降低局部复发率,尤其对那些切端阳性患者。结论腹部MHF侵袭性强,复发率高,病理分级差,以手术切除为主辅以放疗有助于提高患者的远期生存率。     

Pleomorphic liposarcoma of a young woman following radiotherapy for epithelioid sarcoma

A case of a metachronous epithelioid sarcoma and pleomorphic liposarcoma in a young woman is described. The first tumor was an epithelioid sarcoma (ES) with focal rhabdoid features localised in the left calf while the second lesion developed seven years later in the same region was diagnosed as pleomorphic liposarcoma resembling myxofibrosarcoma ( myxoid variant of malignant fibrous histiocytoma ) predominantly composed of moderately differentiated spindle cells. Multiple foci of uni- and plurivacuolated lipoblasts were seen. Following the resection of ES the patient received 57 Gy radiation to the region, therefore we regarded the second tumor as a radiation induced liposarcoma. A further interesting feature of this case is that the development of pleomorphic liposarcoma preceded by 6 months the solitary right parabronchial metastasis of ES and after 4 months of metastasectomy a third tumor developed at the site of the first lesion. This tumor showed dedifferentiation toward pleomorphic malignant fibrous histiocytoma. Our case represents a unique case of postirradiation liposarcoma developed on the base of ES.     

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.     

Myxoid malignant fibrous histiocytoma of the bladder

Although the most common soft tissue sarcoma of adulthood, malignant fibrous histiocytoma (MFH) is an extremely rare tumor of the urinary bladder. Only three well-documented cases have been reported in the world literature. The patient presented in this report represents the first case of the myxoid variant to develop in the urinary bladder. Whereas all previous patients with MFH of the bladder had intermittent hematuria, this patient's chief complaint was bladder outlet obstruction due to extension of the tumor into the prostate. He was managed with radical cystoprostatectomy, postoperative radiation therapy to the tumor bed, and adjuvant chemotherapy using doxorubicin. The patient tolerated the therapy well and was disease-free at the 3-year follow-up visit. The histogenesis, clinical features, pathologic characteristics, and treatment considerations of this rare bladder tumor are discussed in detail.     

Retroperitoneal sarcomas

Opinion statement The approach to the management of retroperitoneal tumors begins with a complete history and physical examination. Imaging of the abdomen and pelvis by computed tomography (CT) provides both an imaging modality and a method by which to obtain tissue for diagnosis. Because a histologic diagnosis is essential in treatment planning, adequate tissue can usually be obtained by a CT-guided core biopsy. If the diagnosis is sarcoma, additional tests necessary for staging include plain chest radiography and evaluation of the liver by either CT scan or magnetic resonance imaging (MRI). The treatment options for primary retroperitoneal sarcomas include chemotherapy, radiation therapy, surgery, or a combination of these modalities; therefore, a multidisciplinary group best manages treatment planning. Primary radiation therapy for cure is seldom effective for retroperitoneal sarcomas but can provide palliation in select cases. Systemic chemotherapy for chemosensitive lesions, such as poorly differentiated liposarcoma, malignant fibrous histiocytoma (MFH), synovial cell sarcoma, and primitive neuroectodermal tumors (PNET), can be useful when used in a neoadjuvant manner. Consequently, surgical resection continues to be the mainstay of treatment for retroperitoneal sarcomas and requires en bloc resection of the primary tumor. Frequently this includes adjacent organs such as colon, small bowel, kidney, adrenal, and pancreas. Postoperative adjuvant therapy with chemotherapy or radiation has not been proven to be of any additional benefit. Overall treatment results are predominantly influenced by tumor stage, grade, size, and margins of surgical resection. Follow-up CT scans at 6-month intervals and surgical resection of recurrences can be valuable.     

Regional chemotherapy with the use of cisplatin and doxorubicin as primary treatment for advanced sarcomas in shoulder, pelvis, and thigh

Eight patients who had large sarcomas in the hip, thigh, or shoulder girdle have been described. Three had osteogenic sarcomas, and one each had Ewing's sarcoma, biphasic synovial sarcoma, pleomorphic liposarcoma, undifferentiated spindling sarcoma, and malignant fibrous histiocytoma. All eight tumors showed evidence of regression after intraarterial infusion of cisplatin and Adriamycin (doxorubicin) given over 48 hours at 3-week intervals, for a total of between three and seven courses. Tru-cut needle biopsy specimens of five of the lesions were normal after chemotherapy. However, after resection of the regressed fibrotic tumor in seven of the patients, four contained foci of probably viable malignant cells. These cell foci were intraosseous in three cases and in the wall of a cyst in one case. In the remaining case, tumor in the distribution of the infused artery regressed, but tumor in a region supplied by an artery that was not infused continued to enlarge. In one patient with osteogenic sarcoma in the pelvis, despite a good response to intraarterial chemotherapy that was followed by surgical resection and radiotherapy, tumor recurred in an adjacent area in tissues supplied by an artery not infused. A hindquarter amputation subsequently was required. With the exception of the two cases in which adequate tumor arterial infusion was not achieved, local primary tumor control was accomplished by intraarterial infusion chemotherapy followed by local resection or radiotherapy and local resection in all patients. Four patients are well without evidence of residual or metastatic sarcoma 3.5 years after presentation in the case of an osteogenic sarcoma of shoulder, 2.5 years after presentation in the case of a large pleomorphic liposarcoma of thigh and groin, 20 months after presentation in the case of lower-thigh malignant fibrous histiocytoma, and 1 year after presentation in a child with an osteogenic sarcoma of lower femur.     

Case report: recurrence of soft tissue MFH in bone due to minute intravenous tumor emboli detected by MRI

We recently encountered a case with local recurrence of malignant fibrous histiocytoma (MFH) in the bone after wide resection, caused by minute intravenous tumor emboli which were retrospectively detected in MR imaging. The patient was a 69-year-old woman who initially noticed a mass in her left thigh. The tumor was diagnosed to be MFH, therefore a wide resection was performed; although the tumor was closely attached to the periosteum, it was not difficult to dissect the tumor subperiosteally from the cortex of the femur. The patient received postoperative brachytherapy, but no chemotherapy. Two years later, the tumor recurred with bony destruction of the femur. We reviewed the pre-operative films obtained by various imaging modalities, as well as the histology of the primary tumor, and found minute intravenous tumor emboli in the MR imaging obtained before surgery. Tumor emboli were also observed histologically in the small vessels of the surgically resected tumor. Such intravenous tumor emboli have recently been implicated in the development of regional bone metastasis near the site of the primary lesion in cases of malignant soft tissue tumors. Therefore, we concluded that the tumor recurrence in our case was caused by small tumor emboli invading the perforating veins of the femur. It is therefore emphasized that MR images should be carefully reviewed for the presence of such intravenous tumor emboli before surgery in cases of high-grade malignant sarcomas. As at the time of writing, our patient remains alive and disease-free, with no evidence of any local recurrence or distant metastasis after wide tumor resection for the recurrent tumor.     

Treatment of malignant fibrous histiocytoma of the heart.

Primary soft tissue sarcoma of the heart is encountered infrequently in clinical practice. Treatment is reported of an intracardiac malignant fibrous histiocytoma, consisting of maximal surgical resection followed by 5600 cGy of conventionally fractionated radiation therapy. Transesophageal echocardiograms done during follow-up were useful in assessing tumor control. Aspects of patient care are discussed in conjunction with a review of the available literature.     

Malignant fibrous histiocytoma: report of five cases and a review of the literature.

Five cases of malignant fibrous histiocytoma are presented and the relevant literature is reviewed. All the patients had locally extensive tumor or eventual recurrent or metastatic disease. Determining malignancy by histologic criteria is difficult. Therefore, histologic, gross, and clinical behavior of the tumor is important. Though the role of radiation therapy and chemotherapy is not yet established, it appears that at least wide "cancer resection," if possible, is the treatment of choice. The unusually high incidence of nonresectable, recurrent, or metastatic disease in retroperitoneal histiocytomas raises the question as to whether all three modalities of therapy should be used in the treatment of tumors at this particular site.     

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.     

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.

     

Postirradiation sarcoma (malignant fibrous histiocytoma) following cervix cancer

A case of postirradiation sarcoma is described. The tumor, a malignant fibrous histiocytoma, occurred in the radiation field 11 years following postoperative external beam radiation therapy (7000 rad) for carcinoma of the cervix. Reports of postirradiation malignant fibrous histiocytoma are rare, and the occurrence of this neoplasm following treatment of cervix cancer has not previously been described. The literature concerning postirradiation bone and soft tissue sarcomas is briefly reviewed, with special attention to malignant fibrous histiocytomas.     

Primary malignant fibrous histiocytoma of the jejunum metastatic to the spine

We report a case of malignant fibrous histiocytoma (MFH) metastatic to the spine. A 41-yr-old male was admitted to our hospital for radiation treatment of MFH of the spine. He began to show signs suggestive of partial small bowel obstruction. Computed tomography demonstated jejuno-jejunal intussusception. The patient was taken to the operating room, where the diagnosis was confirmed. Partial jejunal resection was performed. The lead point of the intussusception was histologically diagnosed to be a high-grade malignant fibrous histiocytoma. We believe that the spinal lesion was the metastatic lesion and that metastasis occurred via the vessels of Adamciewicz. To our knowledge this is the first case thus reported.     

髋臼周围肿瘤切除术后重建

目的探讨对髋臼周围肿瘤手术切除及重建骨盆稳定性的方法，最大限度保留肢体功能，减少复发。方法2002～2005年15例髋臼周围肿瘤患者，其中软骨肉瘤8例，恶性纤维组织细胞瘤3例，Ewing肉瘤2例，转移瘤2例；6例配合化疗，4例行瘤段灭活再植，1例行瘤段灭活再植＋人工全髋置换，10例行人工假体置换（包括全髋）。结果1例术后死亡，1例感染，清创后股骨头旷置；14例术后随访9～33个月，4例软骨肉瘤复发，其他病例均能保留患肢部分功能和骨盆稳定性。结论恶性纤维组织细胞瘤、Ewing肉瘤化疗有效者保肢率高，局部复发率低；软骨肉瘤复发率高；瘤段灭活再植因骨愈合时间长，影响功能；股骨头旷置肢体短缩，跛行明显；人工假体置换可最大限度地保留肢体功能；单纯转移瘤为提高生存质量，减轻痛苦，可考虑手术治疗，但年龄大，全身脏器应急能力差者，手术应慎重。     

Chemotherapy of malignant fibrous histiocytoma of bone. A report of five cases

Five patients with evaluable malignant fibrous histiocytoma (MFH) of bone (three with primary tumor and two with primary tumor and metastatic disease) were treated with preoperative chemotherapy including high dose methotrexate (HDMTX) with citrovorum factor rescue (CFR) as is used for patients with osteogenic sarcoma. All five patients demonstrated a clinical response to chemotherapy. Three of four patients who underwent surgery had complete responses and one patient had greater than 90% tumor necrosis as documented by histologic examination of the resected primary tumor. All four patients who underwent surgery following preoperative chemotherapy are surviving free of disease from one to six years from the start of treatment; chemotherapy was discontinued after six to 11 months in these patients. The median disease-free survival time is 31.5 months. This study demonstrates the effectiveness of chemotherapy in MFH of bone, and in particular the effectiveness of HDMTX with CFR which caused measurable responses in all patients while receiving this therapy as a single agent.     

A case of primary malignant fibrous histiocytoma of the vagina

A 52-year-old woman visited us complaining of a recurrent vaginal tumor, which was diagnosed histologically as malignant fibrous histiocytoma of the vagina. Computed tomography revealed multiple metastatic lesions in the liver, lymph nodes and vertebrae. The patient was treated with combination chemotherapy consisting of cyclophosphamide, adriamycin and cis-platinum, but the tumor did not yield readily to the treatment. We believe that this is the first reported case of this tumor in this location in Japan.     

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.     

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.     

Fibrous tumors of the pleura

Opinion statement Fibrous tumor of the pleura is a rare tumor arising from mesenchymal cells underlying the visceral or parietal pleura. The tumor may have benign or malignant histological features, but these do not always predict the clinical behavior of the tumor. In most cases, the tumor appears pedunculated, and simple resection of the tumor is curative even if significant cellular atypia is present. In contrast, some tumors with a broad base of attachment may recur and occasionally become malignant. Complete surgical resection is the mainstay of therapy for both benign and malignant fibrous tumors of the pleura. When resection is incomplete or impossible, external radiation therapy with or without chemotherapy is recommended.     

Malignant fibrous histiocytoma of soft tissue in childhood

Seven children aged 6 months to 11 years with malignant fibrous histiocytoma, a type of sarcoma of soft tissues, have been treated at the Children's Hospital of Philadelphia from January 1975 through July 1983. The primary tumor arose in the head and neck region in three patients, the chest wall in two patients and the pelvis or buttock in one patient each. Operative management consisted of complete tumor removal in the two patients with chest wall tumors, and biopsy only in the remaining five children. Afterward, all seven patients were treated with a multiple-agent chemotherapy program consisting of vincristine, dactinomycin, and cyclophosphamide for two years, with or without Adriamycin (doxorubicin). The five patients with residual tumor also received radiation therapy (RT) in doses of 1500 to 5500 rad. The two children with localized, completely excised sarcoma are continuously free of tumor at 1.4 and 9 years after initiation of treatment. Of the five with residual sarcoma, three had a complete response to radiation and chemotherapy, and two of them are free of recurrence at 4 and 5 years, respectively. In the three remaining children, the tumor spread regionally into the central nervous system or distantly into the lungs, subcutaneous tissues, and liver. Childhood malignant fibrous histiocytoma of soft tissue appears to be similar to childhood rhabdomyosarcoma in its modes of spread and response to management. Operative removal is the key to successful therapy. The roles of multiple-agent chemotherapy and RT remain to be defined. Adriamycin appears to be the most promising single agent. In the absence of concrete data, it seems prudent to follow the same guidelines for irradiation as those used for other soft tissue sarcomas of childhood.