15例腺泡状软组织肉瘤临床特点及预后分析

背景与目的：腺泡状软组织肉瘤（alveolar soft part sarcoma,ASPS@是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有远处转移（肺转移,脑转移及骨转移等@。本文旨在研究腺泡状软组织肉瘤的临床特点,评估其预后因素。方法：回顾性研究了2003年1月-2008年12月就诊于北京大学人民医院骨与软组织肿瘤治疗中心的15例腺泡状软组织肉瘤患者的临床资料,并随访了他们的预后情况。15例患者中,女性9例,男性6例;小于或等于30岁者8例,大于30岁7例;肿瘤大于5cm者11例,小于5cm者4例;原发于软组织12例,原发于骨骼3例;未见其他部位转移6例,肺转移6例,骨转移3例,脑转移2例;术后局部复发2例;术后9例接受放疗,6例未接受放疗;术后接受化疗11例,未接受化疗4例,化疗方案MAID：多柔比星,氮唏米胺及异环磷酰胺。结果：患者预后情况较差,其中无瘤生存6例,带瘤生存4例,死亡5例,中位生存时间为42.0个月,5年生存率为41.7%。伴有远处转移（肺转移、脑转移及骨转移@的患者5年生存率远低于整体生存率。患者个人性别、年龄对预后无显著性差异。肿瘤大小、部位及有无远处转移是影响预后的重要因素。结论：腺泡状软组织肉瘤是高度恶性软组织肿瘤。尽早评估病情,制定合适的治疗方案,原发病灶采取广泛切除,以降低复发及转移风险。但由于本组数据较少,放化疗是否有效还需进一步研究。     

Histopathological outcome of 597 isolated soft tissue tumors suspected of soft tissue sarcoma: a single-center 12-year experience.

BACKGROUND: The aim of this present report was to analyze the patients referred to us with the presumptive diagnosis of soft tissue sarcoma (STS). METHODS: We reviewed all patients referred to us with suspected soft tissue sarcoma (STS) of the extremities or trunk over a 12-year period. RESULTS: We treated 597 patients with soft tissue tumors. Open biopsy revealed soft tissue sarcoma in 318 cases, benign mesenchymal tumor in 124 cases and isolated metastases (ISTM) from carcinomas in 98 patients; other pathologies were found in 57 patients. The primary carcinomas were lung cancer in 26 patients, breast cancer in 19 patients, renal carcinoma in 16 patients, carcinoma of the esophagus in 12 patients, colonic carcinoma in 5 patients, thyroid gland cancer in 6 patients, and in 14 patients carcinoma of unknown primary was diagnosed. CONCLUSIONS: In our collective with soft tissue tumor, 50% of the patients had the diagnosis of soft tissue sarcoma, 20% presented with a metastasis of carcinoma and 20% had a benign tumor. Referring to our results, in patients with the presumptive diagnosis of soft tissue sarcomas, soft tissue metastasis of a primary carcinoma was unexpectedly common, indicating that greater consideration should be given to this differential diagnosis.     

Alveolar soft part sarcoma metastatic to small bowel mucosa causing polyposis and intussuseption

A report of alveolar soft part sarcoma metastatic to the small bowel is presented. Hematogenous metastases to the small bowel from primary tumors outside the abdominal cavity are uncommon, and most remain asymptomatic and are not discovered until autopsy. However, small bowel metastases can lead to intestinal obstruction, intussuseption or even perforation. While metastases to the small bowel have been described for other tumor types, including melanoma and lung cancer, this is extremely uncommon for sarcoma, especially alveolar soft part sarcoma. We describe a 42-year-old male with a long history of alveolar soft part sarcoma, metastatic to the lung and brain, who developed an intussuseption from metastases to the small bowel.     

Surgical treatment of tendosynovial sarcoma.

Tendosynovial sarcoma is notorious for its high rates of local recurrence and metastases after surgical treatment. A retrospective study was made of 109 cases of monobloc wide soft part resection and amputation for primary tumors, and 29 patients who underwent resection of pulmonary metastases. Actuarial five-year survival rates after soft part resection was 70% for untreated, and 61% for previously treated, locally recurrent neoplasms. Corresponding rates after amputation were 47% and 64%. Local tumor recurrence developed in 18% of the primary soft part resections and 4% of the amputations, usually when some basic surgical principle had been violated. 35% five-year survival was achieved with judicious resection of solitary and multiple lung metastases in most cases without chemotherapy. Local control of a tendosynovial sarcoma can be achieved with properly executed surgical procedures which adhere to established tenets of cancer surgery. Resection of pulmonary metastases merits an important position in the management of these patients.     

Metastatic tumors to the pancreas: The role of surgery

Pancreatic metastases from other primary malignancies are a rare entity. By far, the most common primary cancer site resulting in an isolated pancreatic metastasis is the kidney, followed by colorectal cancer, melanoma, breast cancer, lung carcinoma and sarcoma. Only few data on the surgical outcome of pancreatic resections performed for metastases from other primary tumor have been published, and there are no guidelines to address the surgical treatment for these patients. In this study, we performed a review of the published literature, focusing on the early and long-term results of surgery for the most frequent primary tumors metastasizing to the pancreas. Results for the Literature’s analysis show that in last years an increasing number of surgical resections have been performed in selected patients with limited pancreatic disease. Pancreatic resection for metastatic disease can be performed with acceptable mortality and morbidity rates. The usefulness of pancreatic resection is mainly linked to the biology of the primary tumor metastasizing to the pancreas. The benefit of metastasectomy in terms of patient survival has been observed for metastases from renal cell cancer, while for other primary tumors, such as lung and breast cancers, the role of surgery is mainly palliative.     

癌瘤转移规律的探讨

在 4 0 0例恶性肿瘤尸解中 ,32 1例 (80 3% )癌 ,79例肉瘤 ,其中 6 5例淋巴瘤 ,14例 (3 5 % )为软组织及骨肿瘤 ,肿瘤转移到肝及肺最常见。有 16 3例转移到肺及肝 ,各占 4 0 5 %。肝的转移瘤依次主要来自乳腺、大肠、卵巢、胃及非何杰金淋巴瘤 (NHL)。肺的转移瘤主要来自乳腺、肝、NHL ,胃及卵巢。淋巴结转移主要累及颈部、纵隔及主动脉周围淋巴结。广泛转移的肿瘤是肺癌、胃癌、乳腺癌和淋巴瘤。尸检材料显示 ,宫颈癌、膀胱癌、咽癌及睾丸肿瘤主要是局部侵犯 ,转移并不广泛。本研究显示 ,恶性肿瘤的扩散与转移基本有下列方式 :直接侵袭 :很多肿瘤可能直接累及周围组织。例如胃肠癌 ,癌细胞沿着肌组织间隙进入临近器官。淋巴管扩散 :任何器官或组织的肿瘤细胞可能进入淋巴管而转移到局部或远处淋巴结 ,它是癌的主要转移方式。血道转移 :远处转移在尸检材料中很常见 ,最常累及的器官是肝和肺 ,特别是软组织肉瘤 ,但晚期癌也很常见血道转移。种植性转移 :这也是很常见的转移方式 ,特别是胃癌、大肠癌、卵巢癌等。本研究也显示与癌瘤手术标本对比研究、肿瘤转移与临床分期、肿瘤部位、组织学类型及分化程度等有密切关系     

Breast sarcoma. A clinicopathologic review of 25 cases

Sarcoma of the breast represents less than 1% of primary mammary malignancies; this study reports 25 such cases. The largest group had malignant fibrous histiocytoma (44%), followed by liposarcoma (24%) and fibrosarcoma (16%). Also represented were clear cell sarcoma, neurogenic sarcoma, leiomyosarcoma, and alveolar soft part sarcoma (4% each). Of 19 patients treated by wide local excision or simple mastectomy with or without adjuvant radiotherapy, 11 had local recurrence develop, of which one patient died and nine of the remaining ten had metastases develop. Of the remaining eight patients in this group with no local recurrence, only two had metastases develop. Of the six patients treated by radical or Patey mastectomy, none had local recurrence develop, but two died of metastases. No patient had metastases develop more than 5 years after diagnosis. Regional lymph node involvement with tumor was observed in only one patient (with malignant fibrous histiocytoma) despite regional lymphadenopathy in seven. The overall mortality at 5 years is 64% but does not increase thereafter. The authors' findings suggest that failure to establish local control is associated with a poor prognosis and that wide local excision or simple mastectomy does not provide sufficient clearance to be used as first-line treatment. Excision of the axillary lymphatics and adjuvant radiotherapy are unlikely to be beneficial.     

Biological Study of R24 Mouse Monoclonal Antibody in Patients Undergoing Thoracotomy for Pulmonary Metastases from Soft Tissue Sarcoma

The disialoganglioside GD3 is expressed on the surface of soft tissue sarcoma, malignant melanoma, and other malignant cells and is, therefore, a potential target for therapeutic monoclonal antibodies. Intravenously administered R24, a murine IgG₃ monoclonal antibody to GD3, induces inflammation and tumor regression at sites of metastatic malignant melanoma. R24 5 mg/m² was given intravenously every other day for six doses to 10 patients with pulmonary metastases from a primary soft tissue sarcoma of the extremity for whom thoracotomy was planned. Resected tissue was available from 7 patients. All metastases expressed GD3; however, expression was heterogeneous within tumors, and in no tumor were more than 80% of the cells GD3 positive. A mild to moderate infiltrate consisting of mononuclear cells with T-cell markers was identified around or within pulmonary metastases in 6 patients. Tolerable acute allergic reactions occurred in all patients, but 3 patients had severe chest tightness and bronchospasm that limited the planned therapy. The setting of thoracotomy for metastatic disease provides an ideal system for studies on the pharmacology and biological effects of monoclonal antibodies that target soft tissue sarcoma antigens.     

Bilateral breast metastases from ewing sarcoma of the femur

We report a case of bilateral breast metastases from Ewing sarcoma of the femur. A 40-year-old woman presented with Ewing sarcoma of the left thigh, treated by complete surgical exeresis and chemotherapy. Secondary, a large tumor appeared in the left breast. Bone scintigraphy, chest, and abdominal computed tomographic scan were normal. A breast biopsy found a malignant tumor composed of small round cells consistent with the initial diagnosis. After the first cycle of chemotherapy, a tumor was discovered in the controlateral breast. After 5 cycles, residual tumors persisted in the 2 breasts. Tumor exeresis was performed and found bilateral breast metastases of Ewing sarcoma. Because of the early recurrence of the left breast tumor, segmentectomy of the right breast and left mastectomy were performed. The histopathological analysis confirmed Ewing sarcoma metastases in the left breast. Despite local radiotherapy, the clinical course was marked by lumbar bone metastasis, local chest evolution, and progression of the disease. Metastases to the breast by extramammary malignant neoplasms are unusual. Sarcoma is an extremely rare cause of breast metastases and our case is the first report of breast metastases from Ewing sarcoma.     

Expectoration of a lung metastasis in a patient with colorectal carcinoma

Metastatic disease is present in up to 20% of patients at the time of diagnosis of colorectal cancer. The most frequently involved sites are the liver and the lungs. A rare form of lung metastatic disease is endobronchial metastases, most commonly seen with breast cancer and colon cancer. Their clinical and imaging profile is similar to primary bronchogenic carcinoma. Tumor expectoration is an unusual manifestation of endobronchial metastases (as well as of the primary lung carcinoma). We report the case of a 75-year-old man with known liver and lung metastatic disease from colon cancer who experienced an episode of tissue expectoration. Pathology examination of the expectorated piece of tissue was consistent with colonic adenocarcinoma. Tumor expectoration is a rare event, with < 30 cases reported in the literature. Most of the cases reported include secondary lung malignancies, with renal cell carcinoma being the most common primary site. Endobronchial metastases from colon cancer are rare. Patients with colon cancer can benefit from sputum cytology because this type of metastases is shown to have exfoliative properties. To assess the need for endobronchial management with stent placement or bracytherapy, bronchoscopy should be considered in certain circumstances (the onset of respiratory symptoms, rapid response to chemotherapy, long history of metastatic disease with multiple systemic therapies given, undulating response in tumor, as well as after a reported episode of tissue expectoration).     

Clinical trials of immunotherapy: present status.

This brief review of the more promising clinical trials suggests that immunotherapy is indeed beneficial for selected cancer patients. Because of its limited potency, it should not be used as primary treatment for malignant disease except as local immunotherapy for certain accessible tumors. It is effective for eradication of primary neoplasms of the skin as well as cutaneous metastases of malignant melanoma and breast carcinoma. The most important role for immunotherapy is in combination with other modalities. It may help control occult micrometastases that cause recurrence and death following surgical procedures or irradiation. Results of adjuvant immunotherapy appear promising for malignant melanoma, for carcinoma of the lung, breast, and colon, and for soft-tissue sarcomas. In combination with chemotherapy, immunotherapy appears to prolong remission and survival in acute myelogenous leukemia and in disseminated tumors of the lung and breast. Clearly, immunotherapy is not a panacea for malignant disease, but it could become an important arm in a multimodality attack on cancer.     

Prognostic factors for patients with microsurgically resected brain metastases

BACKGROUND: With the advent of new therapies for metastatic carcinoma to the brain, patterns of intracranial disease and factors influencing survival become important considerations when examining treatment options. This study was conducted at a single institution to determine prognostic factors for tumor response and patient survival after microsurgical resection of brain metastases. PATIENTS AND METHODS: 187 consecutive patients who underwent microsurgical resection of brain metastases between July 1989 and September 1996 were retrospectively reviewed and statistically analyzed. The primary cancers included lung cancer (85), gastrointestinal cancer (20), renal cell cancer (19), breast cancer (17), malignant melanoma (8) and 38 cases of various other carcinomas or of unknown primary site. 111 patients received whole-brain radiation therapy (WBRT) with a mean dose of 32 Gy after tumor resection. The influence of number, size, and localization of brain metastases as well as histology of the primary tumor, preoperative performance status, presence of extracranial systemic disease, time course and adjuvant radiation therapy were statistically evaluated (uni- and multivariate) as prognostic factors for survival. RESULTS: Early postoperative Karnofsky score was improved in 59%, unchanged in 32% and worse in 9% of patients. Median survival time (MST) was 9.8 months (range 1 day-5.3 years). The most important parameter showing a significant influence on survival time was the histology of the primary tumor, with prediction of a bad outcome especially for patients with metastases from renal-cell cancer and malignant melanomas. Patients with breast cancer showed longer survival than patients with other primary cancers. Furthermore, survival varied significantly depending on location of brain metastases, performance status at time of craniotomy and duration of symptoms. None of the other studied variables gained prognostic significance. CONCLUSION: Microsurgical resection of one or more brain metastases followed by WBRT still is a useful and efficient treatment in a carefully selected patient group to prolong median survival and improve or stabilize the performance status. Among the factors determining the survival times in this patient group, histology of the primary tumor is most important, together with preoperative Karnofsky score, location of metastasis and preoperative duration of symptoms.     

Clinical and cell line specific expression profiles of a human gene identified in experimental central nervous system metastases

Some cancers, particularly malignant melanomas and carcinomas of the breast and lung, metastasize to the central nervous system (CNS) in advanced stages. In order to develop into clinically manifest metastases, hematogenously disseminated tumor cells must respond to trophic factors within the CNS microenvironment. We have previously identified a nuclearfactor, com1, expressed in human breast carcinoma cells upon formation of experimental metastatic tumors in the CNS. In the present study distinct com1 mRNA expression was detected in cerebral metastases from patients with lung carcinomas, whereas the expression level was generally much lower in glioblastomas (primary brain tumors). In tissue specimens from normal brain and lung, as well as in glioma and lung carcinoma cell lines, com1 expression was barely detectable. One potential mechanism involved in the induction of com1 expression was indicated in the metastatic MCF7/LCC2 breast carcinoma cells. Significant increases in the level of com1 mRNA were observed upon activation of receptor tyrosine kinase signaling, which is known to operate during metastatic tumor cell proliferation within the CNS. The observations in this study strengthen the assumption that com1 may be involved in the tumor cell response to regulatory signals upon metastasis formation.     

Metastasis to the breast from nonmammarian solid neoplasms

Primary breast carcinoma is the commonest neoplasm in women. Although rare, metastases of solid tumors from elsewhere to the breast may occur. Apart from cross-lymphatic metastasis from contralateral primary breast carcinoma, hematopoietic neoplasms occasionally involve the breast. As far as we know, less than 500 patients with secondary extramammary solid neoplasms involving the breasts have been reported in the English literature, of which malignant melanoma and lung tumors constitute the leading cause. Herein, five additional adult cases are reported and literature is reviewed. Two of the patients had primary rhabdomyosarcomas, two ovarian carcinomas, and one colon carcinoma. In one case with ovarian carcinoma, breast mass was the only manifestations of the disease relapse. All, except one with disseminated disease, had pathological diagnosis. Two of the patients died soon after the detection of breast metastasis. As a result, breast mass can be the first manifestation of relapse or part of a disseminated disease, and usually predicts poor survival.     

Soft-tissue sarcoma: initial characteristics and prognostic factors in patients with and without metastatic disease

Five hundred and sixty-five patients with soft-tissue sarcoma were admitted to Memorial Sloan-Kettering Cancer Center from July 1, 1982 to July 1, 1985. Clinical and pathologic factors were analyzed to identify initial characteristics and to determine factors associated with prognosis and the development of metastasis. In decreasing order of frequency, the most common sites of primary tumor were the extremity (51.1%), retroperitoneum/viscera (28.8%), trunk (15.9%), head and neck (3.7%) and breast (0.4%). The most common histologic types were liposarcoma (21.4%), malignant fibrous histiocytoma (20.2%), leiomyosarcoma (20.0%), fibrosarcoma (11.0%), and tendosynovial sarcoma (9.6%). One hundred and twenty-eight patients (22.7%) had metastases in a median follow-up period of 19 months. Metastasis was more common in patients with retroperitoneal and visceral sarcomas than in patients with extremity sarcomas. Patients with leiomyosarcomas were more likely to develop metastases than those with other histologic types. Fibrosarcoma and liposarcomas were the least likely to develop metastases in this time frame. Tumors greater than 5 cm in diameter occurred more frequently in patients with metastases. Actuarial survival was related to the site of primary tumor and the histologic type and grade of malignancy. Multivariate analysis indicated that patients with low-grade sarcomas, extremity sarcomas, and fibrosarcomas and patients without metastatic disease demonstrated significantly improved survival. In patients with metastases, disease-free interval (less than 365 or greater than or equal to 365 days) was significantly related to overall survival.(ABSTRACT TRUNCATED AT 250 WORDS)     

Metastases to the breast: differential diagnosis from primary breast carcinoma

Eight patients with breast metastases from primary tumors other than breast carcinoma were studied: 3 malignant melanomas, 2 rhabdomyosarcomas, 1 malignant mesothelioma, 1 appendiceal carcinoid, and 1 epidermoid cervical carcinoma. All had mammographic, histopathologic, and immunohistochemical examinations. The main problem was differential diagnosis from primary breast carcinoma. History of extramammary primary tumor was helpful but breast metastasis was the first clinical feature in 2 cases. Patients had noticed palpable, round, rapid growth masses which were mammographically benign. Pathologic diagnosis was difficult and immunohistochemical studies necessary, whenever the proliferation had histologic features of primary breast carcinoma or when no primary tumor was known. However, some histologic features were of value for diagnosis of metastasis: atypical histologic features for a primary breast carcinoma, a well-circumscribed tumor with multiple satellite foci, the absence of an intraductal component, and the presence of many lymphatic emboli. In adults, the most frequent types of tumors metastasizing in the breast are malignant melanoma and neuroendocrine-like tumors, especially small cell carcinoma and carcinoid. In children, rhabdomyosarcoma is the most common. Accurate diagnosis of breast metastasis is important to avoid unnecessary mastectomy and to implement an appropriate systemic therapy.     

Merkel cell cancer of the skin.

Merkel cell carcinoma (MCC) is a rare malignant cutaneous tumor of the elderly with rapidly growing skin nodules found predominantly on sun-exposed areas of the body. The vast majority of patients present with localized disease, while up to 30% have regional lymph node metastases. Despite local excision and the incidence of local recurrence, regional lymph node metastases and distant metastases is high and usually occurs within 2 years of primary diagnosis. The optimal treatment for patients with MCC remains unclear. The best outcome is achieved with multidisciplinary management including surgical excision of primary tumor with adequate margins and post-operative radiotherapy (RT) to control local and regional disease. Patients with regional nodal metastases should be treated with lymph node dissection plus RT. Adjuvant chemotherapy (CT) should be considered as part of the initial management. In case of metastatic disease CT based on regimens used for small-cell lung cancer is the standard treatment of care.     

Cardiac tumors of mice

Of the 7 primary tumors of the mouse heart, accessed at the Registry of Experimental Cancers, 3 were mesenchymal cell sarcomas, 3 were hemangioendotheliomas, and 1 was a mesenchymoma. The mesenchymal cell sarcomas metastasized via the bloodstream to lungs, kidneys, and spleen. Five of the tumors arose in mice treated with ionizing radiation. Previously, only 3 primary cardiac tumors of mice have been reported. Tumors of the heart as part of multifocal neoplasms of a single histologic type were observed in 3 mice; 2 of these tumors were type B reticulum cell sarcomas, and 1 was a reticulum cell sarcoma not otherwise specified. Of the metastatic tumors of the heart, 12 were from alveologenic carcinoma, squamous cell carcinoma, or carcinosarcoma of the lung, 4 were from sarcoma of the subcutaneous soft tissue, and 1 each was from a subcutaneous transplant of a malignant schwannoma and from an alveologenic tumor of the lung.     

Regional lymph node metastases in patients with soft tissue sarcomas of the extremities, what are the therapeutic consequences?

In the period 1977-1983, 183 adult patients with soft tissue sarcomas of the extremities were treated in the Netherlands Cancer Institute. One hundred and seventy-one patients had initially operable tumors. Fifteen patients (8.2%) developed regional lymph node metastases (RLNM) during the course of their disease. Only two patients (1.2%) developed RLNM as first site of tumor recurrence. The incidence of RLNM varied according to the histological subtypes: liposarcoma: 1/64, fibrosarcoma: 1/12, tendosynovial sarcoma: 5/24, unclassifiable sarcoma: 3/8. The outcome in patients with RLNM was invariably fatal. In all cases with RNLM distant metastases were present either at the time RLNM were found or shortly afterwards (median 4 months). Based on this experience we now consider RLNM in soft tissue sarcoma an expression of systemic tumor spread, which should be treated as such. We find no indication for elective lymph node dissection as part of the initial treatment of soft tissue sarcoma of any histological subtype. Therapeutic lymph node dissection might be indicated as part of the palliative management.     

Divergent effects of macrophage toxins on growth of primary tumors and lung metastases in mice

The effects of silica and carrageenan on primary tumor growth and metastases were evaluated in c57bl/6 and BALB/c mice transplanted with the poorly immunogenic Lewis lung (3ll) carcinoma, mFS6 sarcoma and Madison 109 carcinoma spontaneously metastasizing to the lungs. Silica and carrageenan significantly enhanced lung metastases and decreased primary tumor weight in all three experimental models. A similar augmentation of lung secondaries was found after i.v. inoculation of 3LL tumor cells. The effects of carrageenan on primary 3LL tumor growth and metastases were observed also in thymus-deprived animals. The effect of macrophage toxins was studied also in C57BL/6 mice transplanted with the M5076/73A ovarian carcinoma. This tumor spontaneously metastasizes to various abdominal organs, but not to the lung. After treatment with carrageenan, lung metastases were observed, but no effect on secondaries at other anatomical sites or on the primary tumor was detectable. It is suggested that host defense mechanisms impaired by silica and carrageenan may have divergent effects in the regulation of growth of some primary tumors and spontaneous lung metastases.