Deep-seated Sarcomas Research Articles

Primary Intrathoracic Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is defined as a low-grade sarcoma derived from an uncertain cell of origin in the reticular dermis. We report a fibrosarcomatous variant of DFSP (FS-DFSP) that arose primarily in the deep thoracic soft tissue. The patient was a 9-year-old girl who presented with dyspnea and low-grade fevers without a clinically detectable mass or a history of skin lesion. Imaging studies revealed a 10-cm mass entirely confined within the thoracic cavity. Three years after a marginal excision with adjuvant chemotherapy and radiotherapy, the tumor recurred in the paraspinal region. Histologically, the primary and recurrent tumors comprised a high-grade spindle cell sarcoma, with a small component of storiform, low-grade, CD34-positive spindle cells, classic for an ordinary DFSP. The diagnosis of FS-DFSP was confirmed molecularly by the demonstration of a COL1A1-PDGFB fusion by fluorescence in situ hybridization and reverse transcription-polymerase chain reaction analyses. To our knowledge, this is the first documented case of a genetically confirmed deep-seated DFSP without an associated superficial soft tissue or dermal component. The implication of this case on expanding the clinical spectrum of DFSP will have to be elucidated in future studies by applying molecular pathologic tools in deep-seated sarcomas in the proper morphologic context.

Sarcomas of the skin in the elderly

Malignant mesenchymal neoplasms of skin and soft tissues are characterized by an extreme clinicopathologic heterogeneity. Although all sarcomas of deep soft tissues may also arise in superficial locations, there are important clinicopathologic differences. Lipogenic sarcomas represent the most frequent type of sarcomas in deep soft tissues, but purely dermal liposarcomas are exceedingly rare. Vascular and fibroblastic or myofibroblastic sarcomas, however, are much more frequent in dermal location. In contrast with most deep-seated sarcomas, morphologic grading of dermal sarcomas has only a limited importance because most types of dermal sarcoma have a rather favorable clinical prognosis. Important exceptions to this rule are epithelioid sarcoma and cutaneous angiosarcoma, which are aggressive sarcomas with an increased rate of distant metastases and tumor-associated death. Dermal sarcomas arising in elderly patients show some important differences in their incidence and prognosis. Whereas cutaneous angiosarcoma, myxofibrosarcoma, undifferentiated pleomorphic sarcoma of the skin showing morphologic similarities to atypical fibroxanthoma, and cutaneous leiomyosarcoma arise predominantly in elderly patients, dermatofibrosarcoma protuberans, epithelioid sarcoma, and superficially located clear cell sarcoma are seen more often in younger patients.

Effectiveness of Radiotherapy in Myxoid Sarcomas Is Associated With a Dense Vascular Pattern

Surgery and adjuvant radiotherapy (RT) have long been the standard treatment for most deep-seated sarcomas; however, since the randomized trial from the National Cancer Institute of Canada, which described similar local control for pre- vs. postoperative RT, both modalities are now widely accepted. As a group, sarcomas are classified as radiation resistant. The subgroup of myxoid liposarcoma (MLS), a sarcoma with a typical vascular crow's feet pattern, is highly radiosensitive, but a mechanism for this phenomenon is unknown. Here we describe our results with preoperative RT and propose a mechanism explaining the high sensitivity based on the distinctive vascularization pattern of MLS. Between 2002 and 2006, 31 sarcoma patients, including 10 with MLS, underwent preoperative RT at our institute. Resected specimens were histologically evaluated, focusing on classification, grade, and vascularization patterns. Twenty sarcomas showed more than 80% pathologic response after preoperative RT. A pathologic complete response was found in all "pure" MLS specimens after preoperative RT (n = 8). There were no pathologic complete responses in the remaining sarcoma patients (n = 23), although 12 showed 80% to 90% pathologic response. In contrast to the remaining RT-resistant sarcomas, the highly responding specimens contained branching vasculature, partial thrombus formation and inflammation of medium sized arterioles, similar to the vascular changes in MLS. Both MLS and sarcomas with MLS-like vasculature are highly radiosensitive. Radiation sensitivity may be explained by changes in medium-sized arterioles, obstructing the specific crow's feet vascularization and inducing hypoxia with secondary tumor cell death.

Deep-seated sarcomas of the penis

Mesenchymal neoplasias represent 5% of tumors affecting the penis. Due to the rarity of such tumors, there is no agreement concerning the best method for staging and managing these patients. Sarcomas of the penis can be classified as deep-seated if they derive from the structures forming the spongy body and the cavernous bodies. Superficial lesions are usually low-grade and show a small tendency towards distant metastasis. In contrast, deep-seated lesions usually show behavior that is more aggressive and have poorer prognosis. The authors report 3 cases of deep-seated primary sarcomas of the penis and review the literature on this rare and aggressive neoplasia.

Early detection of a deep-seated synovial sarcoma by magnetic resonance imaging

An 18-year-old man who complained of left thigh pain was referred to our institute. No abnormal findings were detected on physical examination and plain roentgenogram or gallium scintigram. On magnetic resonance imaging (MRI), a high-signal of 1-cm diameter was seen in the left vastus medialis muscle on T1- and T2-weighted images. The lesion was initially considered to be: (1) angiolipoma, which is usually associated with fatty tissue, based on the MR findings or (2) a sport-induced inflammation based on the patient's history of baseball playing. Because of the persistent thigh pain and similar findings on the second MRI, an excisional biopsy was performed; the lesion was diagnosed histologically as synovial sarcoma. Additional wide resection was carried out later, without adjuvant therapy. He has been disease-free for 3 years postoperatively. Many soft tissue sarcomas are discovered with extensive tumor formation and are accompanied by obvious abnormal findings on various imaging studies. It is extremely rare that deep-seated sarcomas are discovered without obvious abnormalities. To the best of our knowledge, this is one of the smallest detected deep-seated soft tissue sarcomas. MRI was the most sensitive and useful procedure for detecting the lesion. Physicians should recognize that painful lesions may possibly be synovial sarcoma, even if they are demonstrated as a subtle lesion of 1-cm diameter on MRI.

Fine‐Needle Aspiration Cytology of Soft Tissue Sarcoma: Benefits and Limitations

Purpose. Examine the benefits and limitations of fine-needle aspiration cytology (FNA) used as the definitive diagnostic method before treatment. Method. Review of the 25 year experience at a multidisciplinary musculo-skeletal centre where FNA is the primary diagnostic approach to soft tissue sarcoma in the extremities and trunk wall and the experience of various experts in the field. Results. FNA has several benefits compared with coarse needle or open surgical biopsy. The most important are rapid preliminary diagnosis, no need for hospitalization and anaesthesia, negligible complications and fear for tumour cell spread. With the collected experience gained during the years a reliable diagnosis of sarcoma is the rule in general and specific-type diagnoses are possible in many histotypes, especially when the cytologic examination is supplemented with ancillary diagnostics. The most important limitations are inability to hit small deep-seated sarcoma and some diagnostic pitfalls such as the correct diagnosis of spindle cell neoplasms, variants of benign lipomatous tumours and ‘new soft tissue tumour entities’. Discussion. Optimal use of FNA calls for certain requirements such as centralization, experience in soft tissue tumour cytology–histopathology, the FNA technique and close co-operation between the orthopaedic surgeon and cytopathologist.

Subcutaneous sarcoma. A population-based study of 129 patients

We reviewed 129 patients with subcutaneous sarcoma diagnosed from 1964 to the end of 1985 in a population-based series of sarcoma cases from southern Sweden. The annual incidence was 0.4 per 100,000, comprising 32% of all soft-tissue sarcomas of the extremities or the trunk wall. Compared to deep-seated sarcomas, subcutaneous tumours were half the size at diagnosis, more common in the lower leg and foot, more often malignant fibrous histiocytoma, and of a lower grade of malignancy. None of the low-grade and only 7% of the high-grade tumours recurred locally after wide local excision without radiotherapy. The cumulative five-year survival for all 129 patients was 80%. Multivariate analysis identified only high grade of malignancy and the size of the tumour (greater than 5 cm) as independent prognostic factors. We conclude that systemic or local adjuvant therapy is not generally indicated for subcutaneous sarcoma because of the good prognosis and low local recurrence rate after wide excision.