恶性纤维组织细胞瘤治疗及预后的研究现状

恶性纤维组织细胞瘤(MFH)是最常见的软组织肉瘤,常发生于四肢软组织,其治疗趋势为以手术为主的综合治疗.肿瘤分期、病理类型及治疗方式均与其预后相关.近年来涌现了大量有关MFH治疗及预后的分子生物学方面的研究,为MFH的治疗及预后评价提供了新的前景.     

Radiation therapy in the treatment of malignant fibrous histiocytoma

Between September 1%9, and September 1978, 17 patients with localized malignant fibrous histiocytomas received radiotherapy at The Medical College of Wisconsin Affiliated Hospitals. Of 5 patients who were treated by radiotherapy alone, 2 were locally controlled: a literature survey revealed that local control was obtained in only 2 of 16 patients treated by radiotherapy alone. Of the 12 patients treated postoperatively, 9 were controlled locally, whereas 12 of 14 patients collected from the literature were controlled with postoperative radiotherapy. Local recurrence following resection is reduced from approximately 50% to 20% by post-operative irradiation. Seven of 17 patients (41 %) developed distant metastases: six patients have died, and the remaining 11 have been followed for 12 to 35 months (median, 25 months). Radiotherapy can eradicate small tumor masses, and given postoperatively, appears to lower risk of recurrence.     

Postirradiation malignant fibrous histiocytoma

Three patients who were treated successfully with postoperative external radiation therapy for ovarian carcinoma, endometrial adenocarcinoma and bilateral retinoblastoma respectively developed years later malignant fibrous histiocytoma (MFH) within the irradiated field. MFH is a recently described soft tissue sarcoma known for its dual fibroblastic and histiocytic differentiation resulting in a pleomorphic histologic appearance.     

原发性软组织恶性纤维组织细胞瘤的MRI影像诊断(附15例报告)

目的:探讨原发性软组织恶性纤维组织细胞瘤(MFH)的MRI影像诊断。方法:回顾性分析15例经手术病理证实的原发于软组织的恶性纤维组织细胞瘤患者的影像学资料,15例患者均行MRI检查,有7例行CT检查,8例行增强扫描。结果:原发于软组织恶性纤维组织细胞瘤15例,肿瘤形态多样,以卵圆形、分叶状多见。病变于T1WI呈低信号或等信号,T2WI呈高信号或等信号为主混杂信号影,7例病变内可见低信号之分隔影。13例病变内见坏死或黏液样改变,2例伴出血。明确有包膜3例。增强扫描均呈显著不均匀强化。结论:MRI对原发性软组织恶性纤维组织细胞瘤的诊断具有重要价值,是首选的影像学检查方法。     

原发性软组织恶性纤维组织细胞瘤的MRI影像诊断（附15例报告）

目的：探讨原发性软组织恶性纤维组织细胞瘤（MFH）的MRI影像诊断。方法：回顾性分析15例经手术病理证实的原发于软组织的恶性纤维组织细胞瘤患者的影像学资料,15例患者均行MRI检查,有7例行CT检查,8例行增强扫描。结果：原发于软组织恶性纤维组织细胞瘤15例,肿瘤形态多样,以卵圆形、分叶状多见。病变于T1WI呈低信号或等信号,T2WI呈高信号或等信号为主混杂信号影,7例病变内可见低信号之分隔影。13例病变内见坏死或黏液样改变,2例伴出血。明确有包膜3例。增强扫描均呈显著不均匀强化。结论：MRI对原发性软组织恶性纤维组织细胞瘤的诊断具有重要价值,是首选的影像学检查方法。     

原发性骨恶性纤维组织细胞瘤的影像学诊断

目的探讨原发性骨恶性纤维组织细胞瘤(PBMFH)的影像学改变,以及X线平片、CT及MRI不同影像学检查方法的应用价值。方法总结经病理证实的35例(PBMFH)的影像学表现,回顾性分析X线平片、CT及MRI的影像学特征,探讨其对(PBMFH)的诊断价值。结果(PBMFH)具备全部恶性骨肿瘤的影像学特征,表现呈多种多样。X线平片和CT常表现为偏心性、侵袭性、溶骨性长骨端骨质破坏,并伴超过溶骨破坏范围的软组织肿块,骨膜反应少见。MR影像改变以等T1、等T2信号为主,其中夹杂斑片状、囊状长T1、长T2信号,但缺乏特异性。结论X线平片是首诊骨肿瘤的主要手段;CT及MRI检查的价值则在于显示病灶范围、皮质破坏、内部坏死及致密残留骨或钙化等骨和软组织细微结构变化情况,特别是MRI还可作为监测病变化疗、放疗疗效及术后复发的重要手段。     

Primary malignant fibrous histiocytoma of the thyroid

Aims and methods

To study the clinical features, diagnosis, and treatment of primary malignant fibrous histiocytoma of the thyroid (MFH-T). Treatment and outcome were analyzed retrospectively in a consecutive series of 12 patients with primary MFH-T treated at the Cancer Hospital of the Chinese Academy of Medical Sciences from 1987 to 2007.

Results

All 12 patients underwent surgery; surgery alone was used in four patients. Five patients were given post-operative radiotherapy, and one patient was given pre-operative radiotherapy. Two patients were given post-operative chemotherapy. Five patients had locoregional recurrence, and five had distant metastases in follow-up. Median survival was 9 months. One patient is alive, and has no evidence of disease. Six patients died six months after treatment, and the other four patients died in 10, 14, 18, and 24 months after treatment, respectively. Nine patients died of the disease, and one patient died of cerebral hemorrhage after treatment.

Conclusion

Primary MFH-T is very rare and has a poor prognosis. Although surgical resection of MFH-T is the treatment of choice in MFH-T, the results are unsatisfactory.     

Primary malignant fibrous histiocytoma of the esophagus

A 59-year-old man was admitted to the hospital for dysphagia and fever. Esophagogram and upper gastrointestinal endoscopy revealed a polypoid mass in the midesophagus. Subtotal esophagectomy, cervical esophagogastrostomy, and lymph node dissection were performed. The histological diagnosis of the tumor was malignant fibrous histiocytoma. An area of severe epithelial dysplasia was found in the esophageal mucosa 3 cm under the tumor.     

Primary malignant fibrous histiocytoma of the aorta

A rare case of malignant fibrous histiocytoma of the aorta is reported. Review of the literature revealed that primary tumors of the aorta are exclusively mesenchymal tumors that almost invariably pursue a fatal course.     

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.     

A malignant fibrous histiocytoma of the breast

We have experienced treating a 49-year-old female with a malignant fibrous histiocytoma (MFH) arising from the right mammary gland. Nine months after surgery, lung metastasis occurred but the lung lesion proved to be resectable. Our findings suggested that the primary lesion had originated from a phyllodes tumor. MFH arising from the mammary gland is an extremely rare tumor, and only 12 such cases have been reported including that of our patient.     

Morphology of experimental malignant fibrous histiocytoma

The microscopic structure of tumors induced in rats by subcutaneous injections of 7,12-dimethylbenz(a) anthracene (DMBA) was studied. Most of them (90%) were histologically identical to malignant fibrous histiocytoma (MFH) in humans. This experimental model is useful in MFH morphogenesis and histogenesis studies in connection with the high frequency and synchronicity of tumor development and distinct localization of tumor at the site of injection.     

Primary malignant fibrous histiocytoma of larynx

Primary malignant fibrous histiocytoma is a rare tumour, more so in larynx. A case is described alongwith biological behaviour and therapy.     

CT FINDINGS OF MALIGNANT FIBROUS HISTIOCYTOMA

ＣＴＦＩＮＤＩＮＧＳＯＦＭＡＬＩＧＮＡＮＴＦＩＢＲＯＵＳＨＩＳＴＩＯＣＹＴＯＭＡＤａｉＪｉｎｇｒｕｉ戴景蕊，ＳｈｉＭｕｌａｎ石木兰，ＬｉＧｅｎｚｈｕ李根柱，ＬｕＮｉｎｇ吕宁（ＣａｎｃｅｒＩｎｓｔｉｔｕｔｅ（Ｈｏｓｐｉｔａｌ），ＣｈｉｎｅｓｅＡｃａｄｅ...     

Primary intrathoracic malignant fibrous histiocytoma and angiosarcoma

Primary intrathoracic malignant fibrous histiocytoma and angiosarcoma are rare sarcomas constituting less than 0.2% of lung cancers. The typical imaging appearance is a large, well-circumscribed, non-cavitating, non-calcified, peripheral lung mass without hilar or mediastinal lymphadenopathy. Bronchoscopy and percutaneous needle aspiration are of limited value in differential diagnosis, and thoracotomy is warranted for definitive diagnosis.     

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 the small bowel

Aims: We report herein an additional case of primary malignant fibrous histiocytoma (MFH) in the duodenum and provide a review of the existing literature. Methods and Results: A 61-yr-old Chinese man was admitted to our hospital with symptoms of melena, anorexia, and weight loss. An abdominal computed tomography (CT) and gastrointestinal barium meal examination demonstrated a tumor of the duodenum suggestive of primary malignancy. The tumor was successfully treated by pancreaticoduodenectomy. It was histopathologically and immunohistochemically diagnosed to be a storiform-type primary MFH of the duodenum. There have been a total of 40 cases of primary malignant fibrous histiocytoma of the small bowel documented in the literature including our Chinese cases. Conclusion: Primary malignant fibrous histiocytoma of the small bowel, especially in the duodenum is extremely rare. The final diagnosis is made only after pathological and immunopathological examination of the tumor. The malignant potential of such tumors is high. The prognosis may be mainly dependent on the invasion and metastasis of tumor, while tumor size is irrelevant. The treatment should be surgery if possible. Early surgical intervention may be the best form of management that may offer the patient good result.     

Radiation-induced malignant fibrous histiocytoma of the brachial plexus

Summary Brachial plexopathy is a common and disabling complication in cancer patients most often attributed to metastasis or radiation-induced fibrosis. Occasionally, other rare but potentially treatable causes are found. A 73 year old woman had a left radical mastectomy followed by radiation to the chest wall and axilla 24 years ago. She recently presented with left arm pain, chronic, nonprogressive lymphedema, profound distal arm sensory loss and progressive severe hand weakness. There was moderate atrophy of all intrinsic hand muscles, anesthesia of the hypothenar eminence and 4th and 5th digits, and no adenopathy or palpable mass in the axilla. EMG confirmed a brachial plexopathy. MRI showed loss of tissue planes consistent with radiation fibrosis, but CT showed a discrete mass in the brachial plexus. Open biopsy showed pleomorphic spindle shaped cells with immunoperoxidase stains consistent with malignant fibrous histiocytoma.Radiation-induced malignant fibrous histiocytoma may present with a brachial plexopathy in the absence of a palpable mass and should be considered in the differential diagnosis of brachial plexus lesions in cancer patients. CT scanning through the plexus may be useful when MRI is normal or equivocal.     

肺原发恶性纤维组织细胞瘤的影像表现

探讨肺原发恶性纤维组织细胞瘤（ＭＦＨ）的影像特点,提高术前确诊率。方法对本院７例及文献中４４例共５１例影像资料进行分析。包括胸片５１例,ＣＴ９例及ＭＲＩ１例。结果本院肺原发ＭＦＨ占同期肺癌０．０１％。全组发病中位年龄５５岁。主要症状为咳嗽、血痰及胸痛。随诊４０例中,死因不明５例,复发和（或）转移１５例,复发率为４２．８％（１５／３５）,发生在一年内占８０．０％。肿瘤位于右叶３４例,左叶１７例。周围型４９例,占９６．１％;中心型２例,占３．９％。影像表现：肿瘤呈软组织肿块,瘤体较大,≥５ｃｍ者３５例,占６８．６％。形态呈规则或不规则形。边缘光整或界限清者２９例,占５６．９％;伴有分叶者２２例,占４３．１％;密度均匀者３３例,占６４．７％。少数病灶边缘有毛刺、囊性变及空洞形成。ＣＴ显示病变密度低且有坏死区,周围组织器官受侵。结论肺原发ＭＦＨ的影像表现虽无特征,但瘤体大,边界清,少分叶,少毛刺,密度低,可有别于常见的肺癌和良性肿瘤。ＣＴ及ＭＲＩ检查有助于显示病变内部密度、侵犯范围和转移情况,可提高诊断率。本病变恶性度高,复发转移快,根治切除可有长期生存的可能