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.     

The significance of bone and cartilage formation in malignant fibrous histiocytoma of soft tissue

Malignant fibrous histiocytoma of soft tissues (MFH), a mesenchymal tumor of varied morphologic patterns and cell types, sometimes contains bone and cartilage. Such bony and cartilaginous elements in a pleomorphic MFH may pose a difficult diagnostic challenge. An MFH with bone and cartilage can be distinguished from extraosseous osteogenic sarcoma and chondrosarcoma on the basis of qualitative and quantitative features of the osseous and chondroid elements. Five cases of soft tissue MFH containing bone and cartilage reported here showed: 1) that the bony and cartilaginous elements are mostly metaplastic and tend to be in the pseudo-capsule or fibrous septa of the tumor; and 2) that the bony elements may show a zoning pattern with peripheral maturation similar to myositis ossificans. In a single case, there was a small amount of "tumor osteoid." However, this was focal and lacked the diffuse and finely divided or ribbon-like pattern generally associated with osteogenic sarcoma. The probability that MFH with bone and cartilage is less aggressive than soft tissue osteogenic sarcoma or chondrosarcoma underscores the importance of accurate histologic diagnosis of these tumors.     

Malignant fibrous histiocytoma of the thoracic region

A clinicopathological investigation of five malignant fibrous histiocytoma (MFH) in the thoracic region was performed. They were two cases of metastasis to the lung and 1 each of the primary lesion in the lung, chest wall and sternal region. This tumor usually occurs on the extremities, in the abdominal cavity or the retroperitoneal areas, so instances of its arising in the thoracic region, except for metastatic episodes, are considered to be rare. Tumors in 4 cases were excised surgically, while one was inoperable. On light microscopy, the lesions were found to be composed of two types of neoplastic cells, fibroblast-like cells and histiocyte-like cells, showing the characteristic histologic pattern of storiform and pleomorphic. Electron-microscopically, these two types of cells were discriminated according to the quantitative and quantitative differences of the various intracytoplasmic organelles. The histogenesis of malignant fibrous histiocytoma has been commonly considered to be the histiocytic cell derived from the bone marrow. However, more detailed pathological investigations are necessary.     

Well-differentiated lymphocytic lymphoma. A study of 47 patients with primary manifestation in the lung

Forty-seven cases of primary well-differentiated lymphocytic lymphoma (WDL) of the lung were studied. Diagnosis was based on histologic identification of a lymphangitic pattern of infiltration and monomorphous (homogenous) cytologic composition. Nineteen cases (40%) had ancillary evidence supportive of a diagnosis of lymphoma including simultaneous or subsequent involvement of other organs, monoclonal immunologic markers, or a monoclonal serum gammopathy. The prognosis for the group as a whole was excellent; follow-up (median, 4 years) was available for 33 cases. Only one patient has died of lymphoma. The authors discuss the histologic differential diagnosis of lymphocytic infiltrates in the lung, propose criteria to distinguish reactive from neoplastic lymphocytic lesions, and discuss the significance of monoclonality in the management of these patients.     

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。     

Neoadjuvant Chemotherapy for Osseous Malignant Fibrous Histiocytoma of the Extremity: Results in 18 Cases and Comparison with 112 Contemporary Osteosarcoma Patients Treated with the Same Chemotherapy Regimen

Abstract

Eigthteen patients with high grade malignant fibrous histiocytoma (MFH) of bone and 112 patients with high grade osteosarcoma (OS) of the extremity were treated with neoadjuvant chemotherapy comprised of methotrexate, cisplatinum, doxorubicin and ifosfamide. For the 18 patients with MFH, surgery involved amputation in 2 cases and limb salvage in 16 (89%); the 112 osteosarcoma patients had amputation in 8 cases and limb salvage procedure in 104 cases (93%). The rate of good histologic response to preoperative chemotherapy (90% or more tumor necrosis) was significantly higher in patients with osteosarcoma than in patients with MFH (74% vs 28%; p<0.003). However, at a median follow-up of 38 months (range 25-61), the 3-year event-free survival (EFS) did not differ in the two groups (MFH 77.8%, OS 70.5%; p=ns). In patients with MFH, no local recurrences were registered, whereas in the osteosarcoma group there were 6 local relapses (5.%).

The effectiveness of neoadjuvant chemotherapy in the treatment of osteosarcoma has been assessed during the last 15 years. The results of the present study seem to indicate that, in spite of a usually poor histologic response to preoperative treatment, neoadjuvant chemotherapy is very effective also in MFH of bone.     

Accuracy of fine needle aspiration and core lung biopsies to predict histology in patients with non-small cell lung cancer

Management of advanced non-small cell lung cancer patients is dependent on the histologic diagnosis for both testing and treatment. This study was designed to determine the ability of fine needle aspiration and core biopsies to correctly determine histologic diagnosis in non-small cell lung cancer. Our institutional cardiothoracic surgery database was reviewed for cases of non-small cell lung cancer treated with lobectomy after a preoperative biopsy by CT guidance or bronchoscopy over a 10-year period from 2002 to 2011. The histologic diagnosis of the final lobectomy specimen was compared to the histologic diagnosis from the preoperative biopsy, and the concordance rate was calculated. 119 biopsy specimens from 117 patients were reviewed. Eighty of the 119 biopsies had the same histologic diagnosis as the lobectomy specimen, yielding an overall concordance rate of 67.2 %. Patients with poorly differentiated tumors were at the highest risk of an incorrect histologic diagnosis on preoperative biopsy. Reliance on fine needle aspiration and core biopsies to determine histologic diagnosis in non-small cell lung cancer may put some patients at risk for suboptimal treatment.     

c-erbB-2 protein expression and clinicopathologic features in colorectal cancer

Previous reports showed that breast and gastric cancers overexpressing c-erbB-2 protein have a greater metastatic potential and worse prognosis than tumors in which this protein is not overexpressed. The present study was undertaken to examine the significance of c-erbB-2 protein expression as a prognostic factor in colorectal cancer. Protein expression was examined immunohistologically in colorectal cancer tissue from 149 patients without distant metastasis, from 38 patients with liver metastasis, and from 18 patients with lung metastasis. The c-erbB-2 protein-positive rate was significantly higher in cases with lymphatic vessel invasion in the primary tumor, but it did not correlate with lymph node metastases. Expression of c-erbB-2 did not correlate with any other histologic feature (histologic type, depth of tumor invasion, venous vessel invasion, or the clinical stage). The positive rate in the primary lesion was significantly higher in cases with liver metastasis than in cases without liver metastasis, the positive rate was significantly higher in the hepatic than in the primary lesions. The expression of c-erbB-2 protein in colorectal cancer tissue correlates closely with liver metastasis but not with lymphatic or lung metastases.     

Spindle cell squamous carcinoma of the lung--two case reports

Two cases of spindle cell squamous carcinoma of the lung are presented. Both cases were female heavy smokers aged 73 and 55. The carcinomas, 7.5 and 6.0 cm in diameter respectively, were located at the peripheral portion of the right lower lung lobes. Direct invasion to the diaphragma and metastasis to the chest wall were observed in the first case but no metastasis was observed in the second case. Histologically, both carcinomas showed a biphasic appearance due to the presence of a squamous cell carcinoma component and a spindle cell sarcomatous component with marked cellular pleomorphism, but no neoplastic bone, cartilage or muscle. Transitional area of two components were seen in the second case.     

Preoperative evaluation of the depth of chest wall invasion and the extent of combined resections in lung cancer patients

The optimal extent of a combined resection in patients with lung cancer invading the chest wall remains controversial. To assess whether specific preoperative findings could lead to the precise evaluation of the depth of chest wall invasion and evade en-bloc resection of the chest wall in cases of tumor invasion limited to the parietal pleura, 132 patients with resected lung cancer involving the chest wall were retrospectively surveyed for the preoperative findings, surgical procedures, pathological results, and survival. A pathological examination of the resected specimens showed that 58 tumors had invaded only to the parietal pleura (shallow invasion) and 74 had involved the soft tissue or ribs (deep invasion). A multivariate analysis showed that preoperative CT findings of obvious tumor invasion beyond the parietal pleura (p = 0.005) and complaints of chest pain (p = 0.015) were independent indicators of deep invasion. In patients with lung cancer involving the chest wall, chest pain and/or invading on chest CT suggested that an en-bloc resection was a suitable surgical procedure, because 79% of those patients had deep invasion. On the other hand, in patients without chest pain and invasion on chest CT, an extrapleural approach was recommended at first based on the fact that 63% of them had shallow invasion. In practice, an extrapleural resection was performed in 40 cases and an en-bloc resection in 10 patients with shallow invasion. There was no significant difference in the survival between the two surgical procedures. Therefore, the CT findings of obvious tumor invasion beyond the parietal pleura and/or the presence of chest pain indicate the need to perform an en-bloc resection in patients with lung cancer involving the chest wall. However, in patients without these findings, an extrapleural approach could be initially attempted for chest wall resection, because an en-bloc resection had no survival benefit for patients with shallow invasion.     

Malignant fibrous histiocytoma of the lung

Primary malignant fibrous histiocytoma (MFH) of the lung is very rare. To date, only 32 reports of 63 cases of primary MFH of the lung have appeared in English, excluding tumors arising from the pulmonary arteries and pleura. We describe a patient with primary MFH of the lung who developed brain metastasis and involvement of pulmonary great vessels. In addition, we reviewed previously reported cases to establish the clinical characteristics and most appropriate management of primary pulmonary MFH. When disease is sufficiently limited, complete resection remains the mainstay of treatment.     

Histologic features of bladder cancer in Boston,USA, Manchester,UK, and Nagoya,Japan

Histologic characteristics of bladder cancer in Boston, USA, Manchester, UK, and Nagoya, Japan, were evaluated. In each of these areas broadly-based series of cases were assembled during a collaborative case-control study. The present analysis was based on 589 cases in Boston, 484 cases in Manchester, and 241 cases in Nagoya. A single pathologist reviewed a slide of the primary tumor without reference to identifying information or other data. The primary histologic type of nearly all tumors was transitional-cell, and there was little variation in the proportion of transitional-cell tumors among the study areas. Nor was there much variation in the distribution of histologic grade, the proportion of tumors showing submucosal invasion, or the proportion of tumors with a papillary surface. Age at diagnosis was strongly correlated with histologic grade. The proportion of grade III (most malignant) tumors was about twice as high among patients 80 years of age and over as among those aged less than 50. An apparent association between age and submucosal invasion was explained in large part by the relationships of histologic grade to submucosal invasion and to age. Other histologic features had only weak and inconsistent relations with age. None of the features evaluated showed consistent associations with history of cigarettesmoking or with sex.     

Malignant fibrous histiocytoma developing in irradiated sacral chordoma

Malignant fibrous histiocytoma (MFH), arising at the site of a sacral chordoma 8 years after massive radiotherapy, is described. Initially, the patient received 7000 rad to the sacral area and, on recurrence, 5 years later, an additional 4000 rad. Two years later, a sacral mass was noted again. Biopsy then revealed MFH; chest x-ray showed multiple lung metastases. A combination chemotherapy, consisting of cyclophosphamide, vincristine, adriamycin (doxorubicin), and DTIC, resulted in a 6 month partial response. Subsequently, the patient died because of progressive metastatic disease. At autopsy, 8 years after diagnosis, both the sacral lesion and the lung metastases proved to be MFH, and no residual chordoma was found.     

Lung cancer heterogeneity. Prognostic implications

Although histologic heterogeneity of lung cancer is well recognized, little information is available related to possible effects of this heterogeneity on prognosis. We collected 100 consecutive lung cancer cases, including 35 autopsies and 65 surgical resections, which were extensively sampled (average, ten blocks per case) and analyzed for histologic heterogeneity. Slides were randomized and classified by five pathologists using the 1981 World Health Organization (WHO) classification scheme. Five-year follow-up data were obtained for the surgical cases, and detailed information on staging and survival from time of diagnosis was available in 91 cases. Survival time was analyzed with respect to the patient's age, sex, stage, predominant histologic pattern, and presence or absence of major heterogeneity. The latter is defined as the presence on at least one slide of a major histologic pattern different from that of the remaining slides for that case. The only statistically significant predictor of survival was tumor stage (P less than 0.0001). Heterogeneous tumors appeared to have a worse survival, but this did not reach statistical significance. There was no relationship between survival and predominant histologic pattern (cell type), sex, or age.     

Pyloric gland metaplasia with perineural invasion of the gallbladder: A lesion that can be confused with adenocarcinoma

BACKGROUND: Metaplastic pyloric glands have been described in a variety of organs including the gallbladder, in which they can extend into the muscular wall and serosa. METHODS: Clinical, histologic, and immunohistochemical features of four cases of gallbladder florid pyloric gland metaplasia with perineural and intraneural invasion are analyzed. RESULTS: The patients with pyloric gland metaplasia and perineural and intraneural invasion were all females ages 57-72 years. A preoperative diagnosis of chronic cholecystitis and cholelithiasis was made for all four patients, but a histologic diagnosis of adenocarcinoma was made for two patients and entertained in two others. Macroscopically the gallbladders showed changes usually associated with chronic cholecystitis. No intraluminal masses were observed in any of the gallbladders. The characteristic microscopic features included florid pyloric gland metaplasia, proliferation of medium-sized nerve trunks more prominent in the muscular layer and serosa, and perineural and intraneural invasion by the metaplastic glands lined by cytologically bland cuboidal or columnar mucin-containing cells. At last follow-up all patients were alive and symptom free 1-7 years after laparoscopic cholecystectomy. CONCLUSIONS: Pyloric gland metaplasia of the gallbladder should be added to the long and increasing list of benign epithelial proliferations that are associated with perineural and intraneural invasion. This lesion should not be mistaken for adenocarcinoma of the gallbladder, a misinterpretation that may have serious therapeutic implications. The pathogenesis of this phenomenon is unknown.     

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.     

Pancoast syndrome caused by extramedullary plasmacytoma

A 61-year-old patient had the clinical findings of Pancoast syndrome with shoulder pain, weakness and atrophy of band muscles, and an opacified area in the apical lung field. Unlike most cases with Pancoast syndrome in which the tumors are primary carcinomas of the lung, the present case was shown to have a plasmacytoma involving the apical parietal pleura and the adjacent chest wall. The need for histologic diagnosis before treating patients with Pancoast syndrome is emphasized.     

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     

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.     

An analysis of the impact of pathology review in gynecologic cancer

Purpose: To analyze the impact of pathology review in gynecologic malignancies.

Methods and Materials: For all new gynecologic patients seen between December 2, 1993 and January 4, 1996, we conducted a retrospective chart review to determine if a pathology review by the institute’s consultant pathologist changed the diagnosis, and if so whether the change altered patient management. A total of 514 patients were seen, of whom 120 had cervical cancer, 226 had endometrial cancer, 122 had a primary ovarian or peritoneal malignancy, 9 had a vaginal malignancy, 28 had vulvar cancer, and 9 had a miscellaneous gynecologic malignancy.

Results: On pathology review the diagnosis changed for 200 of 599 specimens (33%). This altered management for 63 of 514 patients (12%). For patients with cervical cancer, the grade of tumor was the main change in pathologic diagnosis, with occasional change in the presence of lymph vascular invasion. These did not translate into patient management alterations. Eight patients (1.5%) had management alterations. The changes in depth of invasion and vascular invasion altered management for 3 patients. Changes in pap smears resulted in two management alterations, and changes in histologic diagnoses altered management for 3 cases. For endometrial primaries the changes in pathologic diagnosis included grade, depth of invasion, and the presence of cervical involvement. This did alter management in 40 cases (8%). For the ovarian malignancies, the main changes were grade, extent of disease, or histologic classification, some of which (10 patients, 2%) resulted in altered management. One patient with a vaginal lesion had the diagnosis changed, which did alter management. Of the patients diagnosed with vulvar cancer, the pathologic diagnosis changed for 11 patients. This included changes in grade and depth of invasion. This altered management of 2 patients. The remaining miscellaneous gynecologic malignancies had only two diagnosis changes that altered management.

Conclusions: Pathologic review of gynecologic malignancies is justified as it can alter patient management. In addition, the process facilitates cooperation of the multidisciplinary team and provides a valuable educational forum to enhance patient care.