Primary malignant fibrous histiocytoma of the lung. A clinicopathologic and ultrastructural study of five cases

Primary sarcomas of the lung are rare malignant neoplasms which have usually been classified as fibrosarcomas or as leiomyosarcomas. Only five cases of primary malignant fibrous histiocytoma (MFH) of the lung have previously been reported in the literature. The authors reviewed 10 cases of primary pulmonary sarcoma of the lung seen at their institution during the last 12 years, and five of these cases met the morphologic criteria for the diagnosis of MFH. The morphology in these cases is identical to MFH of other body locations, and shows a combination of fibroblast-like and histiocyte-like cells at both the light and electron microscopic level. By electron microscopic examination there was no evidence for anaplastic carcinoma, rhabdomyosarcoma, or leiomyosarcoma. Of the three patients with adequate follow-up, one patient is alive without evidence of disease at 10 years, one died at 5 years without evidence of recurrent sarcoma, and one died at 7 months with liver metastasis. Two other patients are alive without recurrence at 8 months and 1 year, respectively, following surgery. Because MFH is primarily a tumor of the soft tissues of the extremities, patients with MFH of the lung must be carefully evaluated to rule out a metastatic origin. The reported prognosis in patients with primary MFH of the lung is poor, but early surgical resection does offer the possibility of a cure.     

Malignant fibrous histiocytoma of the lung

Twenty-two cases of primary malignant fibrous histiocytoma (MFH) of the lung are described, and a review of the literature is presented. As in the soft tissue, this sarcoma is found primarily in older individuals, usually as a solitary peripheral mass. Chest wall invasion at diagnosis was seen in four cases. The most common histologic subtype was the storiform-pleomorphic type of MFH. Vascular invasion was seen in 50% of cases. Histologic features did not correlate with prognosis, and subsequent aggressive behavior was related primarily to symptomatic presentation, advanced stage at diagnosis, incomplete excision, invasion of the chest wall or mediastinum, and subsequent recurrence or metastasis. Primary MFH of the lung should be distinguished from spindle cell carcinoma and inflammatory pseudotumors, and this histologic differential diagnosis is discussed.     

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.     

CT引导下放射性粒子植入治疗恶性纤维组织细胞瘤肺转移瘤临床疗效研究

  目的  研究CT引导下经皮穿刺植入放射性125I粒子治疗恶性纤维组织细胞瘤术后肺转移瘤的临床疗效。  方法  选取自2006年1月至2011年8月在天津医科大学附属第二医院接受治疗的恶性纤维组织细胞瘤术后经病理明确诊断出现肺转移瘤的患者10例, 对转移病灶行CT引导下经皮穿刺肿瘤内放射性125I粒子植入治疗。术后6个月复查胸部CT, 与粒子植入前比较肿瘤变化, 按照国际标准判定疗效。随访自2006年6月至2012年6月。  结果  全部患者顺利完成粒子植入治疗, 靶区接受的平均照射剂量为(207.4±43.1)Gy, D100(99.7±10.6)Gy, D90(127.5±16.1)Gy。植入术后6个月随访, 有效率80.0%。截至2012年6月, 10例患者中2例生存, 8例死亡, 死亡患者术后平均生存时间14.6±3.5个月, 中位生存期13个月。术中6例患者出现气胸, 其中3例行胸腔闭式引流术, 3例行胸腔穿刺抽气; 6例患者出现肺内针道出血, 不伴咳血, 无进行性血胸, 止血处理后症状消失, 1个月后复查出血吸收。  结论  放射性粒子对于恶性纤维组织细胞瘤术后肺部转移病灶短期局部效果明显, 可以作为一种有效的局部治疗手段。      

A case of primary malignant fibrous histiocytoma of the right lung

A malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma found in adults, but a primary MFH of the lung is very rare. A chest X-ray taken of a 73-year-old man revealed a massive lesion in the right upper lobe, and a subsequent right upper lobe lobectomy uncovered a soft, light yellow tumor in the right S3 area, measuring 2.8 x 2.6 x 1.7 cm. Histologically, the tumor manifested a typical storiform pattern of spindle-sharp cells with no sign of a myosarcoma. As no abnormal lesion was detected in the abdomen and extremities, the tumor was diagnosed as a primary MFH of the lung.     

纵隔原发性恶性纤维组织细胞瘤

为了研究原发性纵隔恶性纤维组织细胞瘤的临床病理特点。采用常规石蜡包埋ＨＥ 染色及免疫组织化学方法研究３例ＭＦＨ。３ 例ＭＦＨ 均位于中纵隔,肿瘤界限清楚有包膜或假包膜,术后无局部复发及转移,最长无瘤生存者已达９ ａ。纵隔原发ＭＦＨ的病理特点与软组织ＭＦＨ 相同,但局部复发率及转移率低,提示其预后好于软组织ＭＦＨ。     

Osteogenic sarcoma. Malignant fibrous histiocytoma subtype

A distinctly different entity from the now well-delineated malignant fibrous histiocytoma (MFH) of bone is the MFH histopathologic subtype of osteogenic sarcoma. Although uncommon, recently the authors have encountered six cases of this neoplasm, in each of which the soft tissue component was devoid of bone elements and was microscopically indistinguishable from MFH of bone or soft tissue. Neoplastic osteoid and woven bone were present in the osseous component of each tumor, however. Radiologically, the lesions generally were osteoblastic but focally osteolytic with features typical of osteogenic sarcoma. Pain was the most common presenting symptom. There was no age or sex predilection. Immunocytochemical staining showed strong positivity with alpha-1-antichymotrypsin within malignant bizarre giant cells and occasional neoplastic osteoblasts in five cases. The biological behavior followed a very aggressive course. Four of the six patients developed pulmonary metastases 6 to 12 months after initial surgery; one patient presented initially with pulmonary metastases. Adequate tumor sampling as well as optimal correlation with clinical and radiographic information are required to distinguish the MFH subtype of osteogenic sarcoma from MFH of bone, both being high-grade neoplasms, however.     

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。     

原发性肺淋巴瘤的影像学表现(附4例报告)

目的:探讨原发性肺淋巴瘤的影像学表现,进一步认识和提高对原发性肺淋巴瘤的影像诊断水平。方法:回顾性总结我院确诊的4例原发性肺淋巴瘤的临床资料及影像学表现,并结合文献对其影像学表现特点进行分析。结果:4例原发性肺淋巴瘤的影像学表现为:肺部单发肿块3例,其中1例肿块无分叶,肿块边界模糊伴分叶及毛刺征2例;肺实变并伴结节样多发病灶1例;病灶内见支气管充气征2例,2例合并胸腔积液。结论:原发性肺淋巴瘤的影像学表现多样,缺乏特异性,多表现为肺内单发肿块,常有多种征象并存,可侵犯胸膜,最后确诊需靠病理。     

Malignant fibrous histiocytoma of bone

Malignant fibrous histiocytoma (MFH) of bone is a malignant primary bone tumour that is being increasingly recognised, as more details emerge in the literature regarding its natural history and precise (although sometimes diverse) histological appearance. When the whole bulk of a malignant bone tumour fits the criteria laid down, the designation of MFH seems appropriate. Seven cases of MFH of bone were encountered from a total of 220 primary malignant bone tumours in our files over a 4-year period. The metaphyses of long bones were the most common sites harbouring the tumour, and a wide age range was represented. Amputation was the treatment of choice in all cases. The relevant literature is reviewed.     

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.     

Myxoid malignant fibrous histiocytoma with multiple primary sites

Malignant fibrous histiocytoma (MFH) is one of the most common types of soft tissue sarcomas in adults. The most common location of MFH are the extremities and the trunk, with the most common site for distant metastases being the lung. We describe a case with multiple synchronous sites of myxoid MFH but no lung metastases and presence of abnormalities of 19p13.     

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 lung

Malignant fibrous histiocytoma (MFH) is among the most common soft tissue sarcomas of adult life, but rarely occurs elsewhere. We report an example of primary MFH of the lung and review 15 previously reported acceptable cases with current follow-up information. Histologically, the tumor in our case was pleomorphic with storiform and fascicular areas. Tumor cells showed positive immunostaining for alpha 1-antitrypsin, alpha 1-antichymotrypsin, and vimentin. Stains for desmin, cytokeratin, myoglobin, epithelial membrane antigen, S-100 protein, and lysozyme were negative. Electron microscopic study showed histiocyte-like, fibroblast-like, intermediate, and undifferentiated tumor cells. A variety of methods were used to treat these patients. Two patients survived for 5 or more years, two were alive and well at 8 and 12 months, respectively, two were alive with metastatic tumor at 3 and 18 months, respectively, and ten patients died of tumor, with an average survival of 1 year.     

Malignant change secondary to fibrous dysplasia

Background Malignant change in fibrous dysplasia (FD) is very rare. This study was carried out to establish some characteristic clinical information about this disorder. Methods Four cases with a malignant change in FD out of 128 cases with FD were surgically treated and followed up for a median period of 61.3 months. The mean age of the patients was 39.8 years. Clinical features, radiological findings, and the outcome were analyzed for each of the four cases. Results and conclusion The sites of the lesions were tibia (2 cases), femur (1 case), and rib (1 case). The forms of FD were monostotic in one case and polyostotic in three cases. Radiologically, plain films and computed tomography (CT) showed osteolytic lesions with poorly delineated margins within and/or near areas having a ground-glass appearance. In the osteolytic lesions, simple cystic changes associated with old FD could be excluded by enhanced magnetic resonance imaging (MRI). Histopathologically, two cases were osteosarcoma, one case was malignant fibrous histiocytoma (MFH), and one case was fibrosarcoma. The management of this disease should be decided according to the type of primary high-grade bone sarcoma. One patient, with MFH, was dead of lung metastasis 13 months after surgery. The others are alive without disease.     

Immunohistochemical study of bone GLA protein in primary bone tumors.

METHODS. The immunoreactivity of bone GLA protein (BGP) in primary bone tumors, including osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma of bone (MFH), and giant cell tumor of bone (GCT), was investigated with anti-BGP rabbit serum and peroxidase-antiperoxidase complex. RESULTS. As to intracellular localization, BGP antigenicity was detected in 33 of 35 cases of osteosarcoma and 12 of 25 cases of chondrosarcoma. However, there were no positive findings in all 15 cases of MFH or 20 cases of GCT. In chondrosarcoma, the frequency of positively stained cases increased according to pathologic grading (i.e., 3 of 14 cases of Grade 1, 7 of 9 cases of Grade 2, and 2 of 2 cases of Grade 3). Although the multinucleated cells in MFH or GCT were not immunostained, BGP antigenicity was observed in the multinucleated cells of osteosarcoma (12 of 15 cases). In the matrix of osteosarcoma, BGP immunoreactivity of the tumorous osteoid was observed in 28 of 32 cases. However, in the matrices of chondrosarcoma, MFH, and GCT, BGP immunoreactivity was not observed. CONCLUSION. These results suggest that the immunohistochemical study of BGP is useful for the differential diagnosis of bone tumors.     

骨原发性恶性纤维组织细胞瘤的病理形态及免疫组化观察

报告１２例骨原发恶性纤维组织细胞瘤，占本单位同期骨原发肿瘤的１．３％及骨原发恶性肿瘤的４．７％。以四肢长骨多见。临床及Ｘ线皆易误诊为骨肉瘤和骨纤维肉瘤。该瘤由纤维母细胞、组织细胞组成，并有多少不等的多核瘤巨细胞、灶性分布的泡沫细胞及炎细胞。α１－ＡＴ和ＬＹＳＯ是ＭＦＨ的重要标志物，尤α１－ＡＴ敏感，有助于与骨肉瘤及骨纤维肉瘤鉴别，超微结构提示ＭＦＨ来源于原始间叶细胞．     

Early detection and differential diagnosis of metastatic lung tumor

In general, the prognosis of cases with metastatic pulmonary tumor is extremely poor. However, in cases where primary lesion is well controlled and no distant metastasis except the lung is clinically found, the pulmonary metastasis could be curable through the early detection of metastasis followed by radical surgery equally to the case with primary lung tumor. The candidate of this curable metastasis is the case with of nodular metastasis with a small number. I discuss the X-ray figure, differential diagnosis, diagnostic accuracy of the number of metastasis, and the interval of follow-up care after treatment of primary malignancy.