Soft tissue sarcoma: the predominant primary malignancy in the retroperitoneum

Purpose. In the clinical work-up of a retroperitoneal mass, the diagnosis of soft tissue sarcoma is often not considered. Incidence rates of various malignant and benign retroperitoneal tumours were studied to determine the incidence of soft tissue sarcoma in comparison with other neoplasms in the retroperitoneal space.Method. Nation-wide data on retroperitoneal tumours, collected prospectively over a 5-year period (1 January 1989- 1 January 1994), were supplied by the Netherlands Cancer Registry and The Dutch Network and National Database for Pathology.Results. Seven hundred and six patients with a primary retroperitoneal neoplasm were identified; 566 patients had a malignant tumour (80%). A soft tissue sarcoma (STS) was the most frequently diagnosed malignant tumour (n = 192), The agestandardised incidence of retroperitoneal STS was 2.5 per million person-years. The male/female ratio for STS was 0.73. In females, STS comprised 41%of all malignant retroperitoneal tumours, carcinoma of unknown primary tumour site (CUP) comprised 31%, and malignant lymphomas (ML) comprised 22%, whereas in males these values were 28% (STS), 30% (CUP), and 32% (ML), respectively.Discussion. Soft tissue sarcomas, albeit rare, are relatively common primary tumours in the retroperitoneum, especially in women.     

Soft tissue carcinoma in the retroperitoneum: an often neglected diagnosis.

AIMS: Successful surgical treatment of patients with retroperitoneal soft tissue sarcoma (RSTS) is based on pre-operative planning that starts with a correct pre-operative diagnosis. In a population-based study, we determined which patients were initially treated for assumed other conditions. The effect of an erroneous diagnosis on the installed treatment was analysed. METHOD: With the help of the Dutch Network and National Database for Pathology (PALGA), data were collected on 143 patients in the Netherlands in whom a primary RSTS was found and confirmed histologically between 1 January 1989 and 1 January 1994. Satisfactory clinical information was obtained on 138 patients, 64 males and 74 females (54%). The median age was 60 (range 18-88) years. RESULTS: At the time of actual treatment 37% of the patients with RSTS were assumed to have another disorder (group 1 n=51), whereas 87 patients were diagnosed as having RSTS (group 2). In group 1, an acute presentation was more common (18 vs 2% P=0.002), and the tumour was less often palpable at physical examination (43 vs 69% P=0.004), while clinical work-up less frequently included CT-imaging (57 vs 89% P<0.001) and a biopsy (29 vs 77% P<0.001). Although tumours in group 1 were smaller (median diameter 13 vs 19 cm P<0.05), this was not reflected in a better operative result: less patients underwent complete tumour resection (51 vs 57%) and more patients underwent surgery for tumours that proved to be irresectable (14 vs 1% P=0.004). CONCLUSIONS: (1) More than one-third of patients with RSTS are misdiagnosed and inappropriately treated; and (2) biopsies and cross-sectional imaging improve diagnosis.     

Management of recurrent soft tissue sarcoma of the retroperitoneum

Local recurrence is the determinant of tumor-related mortality in retroperitoneal sarcomas because death often occurs as a result of local progression mostly without synchronous metastasis. Complete resection of the lesion and surgical margins status are the only therapeutic factors significantly associated with local control. Further outcome improvements need multimodal therapy since prognosis of these recurrences is poor with lower rates of complete resection and higher grade of malignancy than primary. Complete resection of the recurrence often requires removal of adjacent organs to achieve negative margins. External beam radiotherapy (EBRT), eventually associated with a boost of intra-operative electron beam radiotherapy (IORT) could improve the outcome in these patients. Preoperative timing could limit its toxicity. Chemotherapy protocols may enhance local and systemic outcome and can reduce the volume of high grade tumors and therefore allow a higher rate of complete resection. Isolated pelvic perfusion with local high doses chemotherapy is under investigation. Surgical excision of lung metastases should remain the treatment of choice, if preoperative evaluation indicates that complete clearance of the metastases is possible. Intra-operative chemotherapy after cyto-reductive surgery for the treatment of sarcomatosis is disappointing and complete surgery remains the cornerstone of the treatment with best results for low grade sarcomatosis. Adequate management at the time of primary presentation is likely to afford the best chance for long-term survival.     

Soft tissue angiosarcomas

From 1949 to 1979, 12 patients with soft tissue angiosarcoma received radiotherapy (alone or in combination with other modalities of treatment) with curative intent at The University of Texas M.D. Anderson Hospital and Tumor Institute. The primary site was the head and neck in six patients (scalp, four; maxillary antrum, one; and oral tongue, one), the breast in four patients, and the thigh in two patients. All four patients with angiosarcoma of the scalp had advanced multifocal tumors, and two of them had clinically positive neck nodes. None of these tumors were controlled locally, and local recurrences occurred within and/or at a distance from the generous fields of irradiation. The remaining two patients with head and neck lesions had their disease controlled by surgery and postoperative irradiation. Three of the four angiosarcomas of the breast were primary cases which were treated by a combination of surgery (excisional biopsy, simple mastectomy, radical mastectomy) and postoperative irradiation. One patient also received adjuvant chemotherapy. The fourth patient was treated for scar recurrence after radical mastectomy. All four patients had their disease locally controlled, and two of them have survived over 5 years. The two patients with angiosarcoma of the thigh were treated by conservative surgical excision and postoperative irradiation. One patient had her disease controlled; the other had a local recurrence requiring hip disarticulation and subsequent hemipelvectomy for salvage.     

Soft tissue sarcomas

Sarcomas are a heterogeneous group of rare tumors that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body, particularly the trunk, retroperitoneum, or the head and neck. Pretreatment radiologic imaging is critical for defining the local extent of a tumor, staging the disease, guiding biopsies, and aiding in diagnosis. Core-needle biopsy is the preferred biopsy technique for diagnosing soft tissue sarcomas. The American Joint Committee on Cancer (AJCC) staging system for soft tissue sarcomas is based on histologic grade, the tumor size and depth, and the presence of distant or nodal metastases. Despite improvements in local control rates with wide local resections and radiation therapy, metastasis and death remain a significant problem in 50% of patients who present with high-risk soft tissue sarcomas. The most common site of metastasis is the lungs, and metastasis generally occurs within two to three years after the completion of therapy. Progress in the molecular characteristics of these tumors should in the near future translate into molecularly based therapies that can be incorporated into standard treatment strategies.     

Soft tissue ameloblastoma

Two cases of oral soft tissue ameloblastoma are described and the relevant literature reviewed.     

Soft tissue sarcomas of the extremity

In 1955, in a series of abstracts entitled "A Half Century of Effort to Control Cancer," Pack and Ariel wrote, "in each individual case, there is a constant battle of judgement between the wisdom of more radical amputation and the need for preservation of that important functional part." This dilemma remains with us today. In his paper on the histogenesis of tumors, Stout, relating to the philosophy of treatment, wrote that "the best chance of curing malignant tumors of the soft tissues lies in the hands of the therapist who makes the first attempt." Pack's words are as true today as they were in 1955, and although limb salvage is possible for many patients with soft tissue sarcomas, preservation of an extremity at the risk of patient survival is not a feasible option. Most efforts at less than amputative surgery must entail multimodality therapy. Although there are some reports of success with single-agent therapy, such as surgery alone, the majority of successful, nonamputative series include surgery with radiation and/or chemotherapy. It must be emphasized that an extremely important aspect of the treatment of these patients is the judgment and evaluation by the primary physician. Therefore it is extremely important that the primary physician be familiar with the many factors involved in prognosis, both in terms of local control and of systemic illness, in order for the patient to be informed of treatment options. If this is not possible, the patient should be referred to a center that is involved in active research protocols or treatments. Although rare, the soft tissue sarcomas remain among the most difficult to treat, even though over the years there has been significant progress in diagnosis, classification, and successful local control. Patient survival is based on a multitude of factors that include the histogenesis of the tumor, its grade, size, anatomical location, the surgical procedure performed, the use of preoperative or postoperative radiation, and the clinical stage of the disease--whether it is primary or metastatic. In time, it is probable that additional factors will be found.     

Soft tissue sarcomas involving the pelvis

BACKGROUND AND OBJECTIVES: Soft tissue sarcomas (STS) of the true pelvis are rare tumors and there is little information in the literature related to pelvic STS. The purposes of this review were to understand the anatomic extension of these tumors to better plan surgical treatment and to determine the outcome of these patients. METHODS: Eighteen consecutive patients presenting between 1987 and 1995 with soft tissue sarcomas involving the true pelvis were retrospectively reviewed at minimum follow-up of 18 months. Cross-sectional imaging was reviewed for each patient to determine the anatomical location of the lesions. RESULTS: The tumors were confined to the true pelvis in 4 patients, extended to the retroperitoneum in three cases, and extended to the thigh in 11 patients. Adjuvant radiation was administered to all but 2 patients who had received radiation to the region in the past and all patients underwent surgical resection (local resection in 13 patients and hindquarter amputation in 5 patients). Surgical resection had a high rate of morbidity and complications including positive resection margins in nine individuals. Of the 18 patients, 11 died at a mean time of 15.5 (2-58) months from surgery, 4 were alive with evidence of disease at a mean time of 44.3 (18-68) months, and 3 were alive with no evidence of disease at a mean time of 57 (43-71) months. CONCLUSIONS: Soft tissue sarcoma of the pelvis is fortunately a rare disease with a high risk of local and systemic disease progression despite treatment with irradiation and surgical resection.     

Soft tissue sarcoma of the pleural cavity

Seventeen cases of soft tissue sarcoma (STS) developing in the pleural cavity were collected from Japanese hospitals, and their clinical and pathologic findings summarized. Eight of the 17 patients had a 15-year to 50-year (mean, 28.8) history of chronic pleural inflammatory disease (pleuritis, pyothorax, and pulmonary tuberculosis) before the onset of the pleural sarcoma. Histologically, malignant fibrous histiocytoma was the most common tumor type (11 cases), followed by angiosarcoma (four). The age at diagnosis of the sarcoma ranged from 15 to 74 years (mean, 58); the male-to-female ratio was 3.3:1. In the eight cases of sarcoma associated with chronic pleural inflammatory disease, male preponderance was more marked (7:1). The commonest presenting symptom was chest pain. A mass could be detected by chest roentgenograms in 13 patients and computed tomographic scans in 15 patients. No patient had distant metastases at first admission. Thirteen patients were treated by surgery, chemotherapy, and/or radiation therapy. Thirteen of the 17 patients died 1 to 87 months (mean, 14.2) after therapy for STS. The actuarial 1-year survival rate was 38.5%. These findings suggest that long-standing pleural inflammation might be an etiologic factor for development of pleural STS.     

Soft tissue aspiration cytopathology

BACKGROUND: Fine-needle aspiration (FNA) biopsy as a diagnostic modality for the pathologic evaluation of soft tissue neoplasms and non-neoplastic soft tissue mass lesions is uncommon and controversial. This procedure contrasts with more traditional diagnostic methods such as marginal excision, incisional (open) biopsy, or even core biopsy to procure tissue from somatic sites. METHODS: The authors reviewed the results of cytopathologic diagnoses obtained by fine-needle aspiration biopsy over a consecutive 11-month period in patients that presented primarily with a palpable soft tissue mass. A few patients with deep non-palpable soft tissue masses also were evaluated by radiologically guided FNA. Cytopathologic diagnoses were verified by different means including tissue examination either by concurrent cell block or subsequent surgical biopsy, flow cytometry, clinical outcome, or repetition of the FNA procedure. Patients were followed for a minimum of one year to evaluate the mass clinically, to determine whether any further therapy was administered, and to assess disease status. RESULTS: Eighty-two aspirates were performed without complications from 77 patients ranging from 12-88 years of age (mean = 50 yrs.) with men outnumbering women 1.5:1. Soft tissue masses were most common in the extremities (41 cases), followed by the trunk (34 cases), retroperitoneum (5 cases), and head and neck (2 cases). Fine-needle aspirates were diagnosed as malignant in 42 (51%), benign in 32 (39%), nondiagnostic in 6 (7%), and atypical in 2 (2%) cases. Malignant aspirates were comprised of 24 sarcomas (57%), 9 carcinomas (21%), 6 malignant lymphomas (14%), and 3 melanomas (7%). Twenty-two aspirates (52%) had an initial diagnosis of malignancy, whereas 18 (43%) represented metastatic and 2 (5%) recurrent neoplasms. Confirmation of the cytopathologic diagnosis was by concurrent or subsequent tissue examination in 57%, flow cytometry in 5%, clinical outcome in 34%, and repeat aspiration in 4%. One false negative and no false positive diagnoses were issued for a sensitivity and specificity of 100% and 97% respectively in distinguishing benign and malignant lesions by FNA. Of the malignant aspirates, 83% could be subtyped whereas 72% of benign aspirates were correctly subtyped. For primary soft tissue sarcomas, 12 of 19 (63%) were accurately subtyped. In 48% of cases a concurrent cell block was obtained and found diagnostically useful in 54% of them. CONCLUSIONS: Aspiration cytopathology of soft tissue mass lesions using FNA biopsy can be an accurate and minimally invasive method for the initial pathologic diagnosis of primary benign and malignant soft tissue masses, for the pathologic confirmation of metastatic tumors to soft tissue, and for the documentation of locally recurrent soft tissue neoplasms. FNA cytopathology is capable of specifically subtyping a large percentage of primary and metastatic soft tissue tumors if cellular material either in the form of a cell block or flow cytometry is obtained in addition to cell smears.     

Soft tissue sarcomas of the adult thoracic wall

Forty-nine adult patients with soft tissue sarcomas of the thoracic wall have been treated by the Division of Surgical Oncology at the University of Illinois. Fibrosarcoma was the most common histologic type (20%). Patients with dermatofibrosarcoma protuberans had the longest mean overall survival time (150.8 months). The mean overall survival time for all patients was 98.2 months, and the disease-free 2-, 5-, and 10-year survival rates were 68%, 51%, and 34%, respectively. Wide excision was the treatment of choice; selected patients also received adjuvant radiotherapy, chemotherapy, or both. In all 10-year survivors, the sarcomas were low grade and well differentiated, or were less than or equal to 5.0 cm in diameter. Aggressive treatment afforded these patients good long-term results.