[NON-VISCERAL NEOPLASMS OF SOFT TISSUES].
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Biopsy techniques for skin, soft-tissue, and bone neoplasms.
This article discusses important aspects of the biopsy of neoplasms in skin, soft tissues, and bone. There are a variety of clinical conditions in which specific biopsy techniques are indicated. Inappropriate biopsy of tumors in these sites may compromise subsequent definitive therapy.
Biopsy↗
Myopericytoma of the thoracic spine: a case report.
STUDY DESIGN: A case of myopericytoma of the thoracic spine is reported. OBJECTIVE: To report a recently described and extremely rare soft tissue neoplasm in the previously unreported location of the thoracic spine. SUMMARY OF BACKGROUND DATA: Myopericytoma is a recently described soft tissue neoplasm with perivascular myoid differentiation. All cases have been reported in the subcutaneous and superficial soft tissues of the extremities. This represents the first reported case of this unusual lesion in the spine. METHODS: A patient with a 3-month history of progressive weakness of the arms and legs was found to have a lytic lesion of T3. The lesion was surgically treated by curettage followed by a course of radiation. Presenting symptoms, imaging studies, histologic findings, and related literature are reviewed in this study. RESULTS: Histologic evaluation of sections of the surgically removed lesion revealed a concentric, periluminal proliferation of cells with prominent positive smooth muscle actin immunoreactivity. These findings are consistent with the diagnosis of a myopericytoma, a lesion not previously reported in the spine. CONCLUSIONS: This study shows the presence of a lesion in the thoracic spine only previously described in the soft tissue of the extremities. The diagnosis of myopericytoma should be included, along with hemangiopericytoma, in the differential diagnosis of lytic lesions of the spine.
Aged↗
Papillary endothelial hyperplasia presenting as a chest wall neoplasm.
Soft tissue hematomas generally resolve but may persist and develop into slow-growing, organized masses. These chronic expanding hematomas are characterized by a pseudocapsule and a predominantly necrotic central cavity, with foci of newly formed capillaries. These have been called chronic expanding hematomas or Masson's papillary endothelial hyperplasia. These lesions can mimic vascular neoplasms and must be considered in the evaluation of expanding soft tissue vascular malformations.
Chronic Disease↗
Practical application of molecular genetic testing as an aid to the surgical pathologic diagnosis of sarcomas: a prospective study.
The strong correlation of specific reciprocal translocations with individual tumor types and the demonstration that polymerase chain reaction (PCR)-based methods can detect translocations in tissue samples have stimulated interest in the role of molecular genetic testing in diagnostic surgical pathology. To evaluate the clinical utility of PCR-based molecular analysis of soft tissue neoplasms in routine surgical pathology, 131 consecutive soft tissue tumors submitted for molecular genetic testing at a tertiary care teaching hospital were prospectively analyzed over a 36-month period. RT-PCR was used to test tumor RNA for fusion transcripts characteristic of malignant round cell tumors (including Ewing sarcoma/primitive neuroectodermal tumor, desmoplastic small round cell tumor, and alveolar rhabdomyosarcoma), spindle cell tumors (including synovial sarcoma and congenital fibrosarcoma), and fatty tumors (myxoid liposarcoma). DNA sequence analysis was used to confirm the identity of all PCR products, and the PCR results were compared with the histopathologic diagnosis. We found that sufficient RNA for RT-PCR-based testing was recovered from 96% of the 131 cases and the percentage of tumors that tested positive for the associated characteristic fusion transcript was in general agreement with those reported in the literature. DNA sequence analysis of PCR products identified both variant transcripts and spurious PCR products, underscoring the value of product confirmation steps when testing formalin-fixed, paraffin-embedded tissue. Only in rare cases did testing yield a genetic result that was discordant with the histopathologic diagnosis. We conclude that PCR-based testing is a useful adjunct for the diagnosis of malignant small round cell tumors, spindle cell tumors, and other miscellaneous neoplasms in routine surgical pathology practice.
Base Sequence↗
MR characterization of post-irradiation soft tissue edema.
OBJECTIVE: Radiation therapy is often used to treat bone und soft tissue neoplasms, and commonly results in soft tissue edema in the radiation field. However, the time course, distribution and degree of this edema have not been well characterized. Our study was carried out to better define these features of the edema seen following neutron and photon radiation therapy. DESIGN AND PATIENTS: Two hundred and twenty-six patients underwent radiation therapy as part of combined modality management for musculoskeletal sarcomas between 1985 and 1993. Of these, 15 had surgical resection of their neoplasm, had no clinical evidence of recurrent disease, and had adequate MR follow-up that allowed sequential assessment of soft tissue following irradiation. Ten patients received photons with an average dose of 52.8 Gy. Five patients received neutrons with an average dose of 17.3 nGy. Sequential MR follow-up was available in these patients for an average of 22.8 months following radiation therapy. On each of the serial MR imaging studies, subcutaneous fat, muscle, and the intramuscular septa/fascial planes were graded subjectively as to size and signal intensity. RESULTS: In general, soft tissue signal intensity in the radiation field initially increased over time, peaking at about 6 months for neutron-treated patients and at about 12-18 months for photon-treated patients. Signal intensity then decreased slowly over time. However, at the end of the follow-up period, signal intensity remained elevated for most patients in both groups. Signal intensity in a particular tissue was greater and tended to persist longer on STIR sequences than on T2-weighted sequences. Survival analysis of signal intensity demonstrated much longer edema survival times for neutron-treated patients than for photon-treated patients. Signal intensity increase in the intramuscular septa persisted for much longer than for fat or muscle. A mild increase in size was noted in the subcutaneous fat and intramuscular septa. Muscle, on the other hand, showed a decrease in size following treatment. This was mild for the photon-treated group and more marked for the neutron-treated group. CONCLUSIONS: There is a relatively wide variation in the duration and degree of post-irradiation edema in soft tissues. This edema seems to persist longer in the intramuscular septa than in fat or muscle. Although the duration of follow-up was limited, our study suggests that this edema resolves in roughly half the photon-treated patients within 2-3 years post-treatment and in less than 20% of neutron-treated patients by 3-4 years post-treatment. Muscle atrophy was seen in both photon- and neutron-treated patients, but was more severe in the neutron-treated group.
Adipose Tissue↗
[Perivascular neoplasms of skin and soft tissues. A review].
Perivascular neoplasms of skin and soft tissues comprise a group of benign and malignant mesenchymal neoplasms showing a perivascular myogenic differentiation. Whereas glomus tumour including its variants represents a clearly defined clinicopathological entity, the whole concept of haemangiopericytoma has been questioned in the last years, and no clear diagnostic criteria are established at the moment. Myopericytomas and myofibromas represent a morphological spectrum of perivascular spindle-shaped lesions. Infantile myofibroma/infantile myofibromatosis and solitary myofibroma of adults are characterized by a biphasic growth of mature, spindled, myofibroblastic cells and smaller, immature, mesenchymal cells associated with numerous haemangiopericytoma-like vessels. Benign and rare malignant myopericytomas of skin and soft tissues are characterized by a concentric, perivascular growth of plump spindled and round tumour cells staining positively for muscle actin, alpha-smooth muscle actin and more rarely for desmin. PEComas represent an enigmatic family of neoplasms composed of perivascular epithelioid, clear, and spindled tumour cells characterized by a coexpression of melanocytic and myogenic markers.
Diagnosis, Differential↗
Small cell osteosarcoma of the soft tissue.
The lesion we described is an osteoid producing mitotically active spindle cell soft tissue neoplasm, which has light and ultrastructural features of an osteosarcoma. By immunohistochemistry it marks with p30/32MIC2-directed antibodies but fails to react with antibodies associated with other soft tissue neoplasms. The precise histogenesis of the lesion is unknown, but failure to elicit a periosteal reaction makes the periosteum less likely to be the primary tissue of origin. The failure to demonstrate any radiologic or histologic evidence of involvement of the underlying bone leaves the soft tissue as the most reasonable site of origin, although the parosteal region cannot be completely excluded.
Adult↗
Magnetic resonance imaging of the musculoskeletal system. Part 9. Primary Tumors.
Magnetic resonance imaging, because of its exquisite soft tissue contrast, has dramatically improved the ability to preoperatively stage primary osseous and soft tissue neoplasms. This technique also has allowed the monitoring of the effects of chemotherapy and the screening for recurrence of neoplasms. The role of magnetic resonance imaging in the preoperative evaluation of the patient with a suspected primary osseous or soft tissue neoplasm is outlined, instances where magnetic resonance imaging potentially may make a specific diagnosis are outlined, the importance of gadolinium enhancement as an adjunct to native magnetic resonance imaging is stressed, and an algorithm for followup of patients after chemotherapy or definitive surgical treatment is presented. In all cases, the magnetic resonance images should be correlated with the plain film, which is still an important aspect of the diagnosis of osseous lesions.
Biopsy↗
Myxoid solitary fibrous tumour of the axilla.
Mostly occurring in pleura, solitary fibrous tumour is an unusual soft tissue neoplasm. Solitary Fibrous Tumour with abundant myxoid stroma represents a rare morphologic subtype of the already uncommon tumour. We report a myxoid solitary fibrous tumour of the soft tissue in a 42-year-old man. The patient presented with a large, movable, nontender mass at the right axilla. The MRI showed a well-defined tumour with increased signal after gadolinium injection. During surgery, the mass was found to be closely associated with the axillary vein. The tumour was completely excised. Pathological and immunohistochemical examinations identified the mass as a myxoid solitary fibrous tumour. The patient was free of tumour recurrence at 18-month follow-up. The rare tumour in an uncommon location makes the diagnosis difficult. Complete excision and long-term follow up are recommended.
Adult↗
Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity.
Proximal-type epithelioid sarcoma is a recently described rare soft tissue neoplasm. It is commonly found in the pelvic, perineal, and genital areas in middle-aged or older adults, as compared with the classic type of epithelioid sarcoma, which arises in the distal portion of the extremities in adolescents and young adults. Proximal-type epithelioid sarcoma has a more aggressive clinical behavior than the classic type of epithelioid sarcoma. Proximal-type epithelioid sarcoma is histologically characterized by a diffuse proliferation of epithelioid cells with prominent rhabdoid feature. Recently, a few cases have been reported of epithelioid sarcoma with elevated serum CA 125 level and CA 125 immunoreactivity in neoplastic cells. These cases raise the possibility that serum CA 125 and CA 125 immunoreactivity could be a useful tumor marker for diagnosing and monitoring epithelioid sarcoma. We describe a case of proximal-type epithelioid sarcoma with elevated serum CA 125 level (up to 3395 U/mL [reference range, <35 U/mL]) in a 12-year-old girl who presented with a huge pelvic mass measuring 12 cm in greatest dimension. The serum CA 125 level dropped to 452 U/mL after a debulking operation of the mass. Immunostaining for CA 125 demonstrated a positive immunoreactivity in the neoplastic cells. She received one cycle of chemotherapy and died of the disease 2 months after diagnosis. This case represented a rare example of proximal-type epithelioid sarcoma with elevated serum CA 125 and immunoreactivity for CA 125 in the tumor cells. Based on the previous reported cases and the current case, serum CA 125 as well as immunohistochemical stain for CA 125 may be a useful tumor marker of proximal-type epithelioid sarcoma.
Biomarkers, Tumor↗
CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: Report of the Radiology Diagnostic Oncology Group.
PURPOSE: To assess the relative accuracies of computed tomography (CT) and magnetic resonance (MR) imaging in the local staging of primary malignant bone and soft-tissue tumors. MATERIALS AND METHODS: At four institutions, 367 eligible patients (aged 6-89 years) with malignant bone or soft-tissue neoplasms in selected anatomic sites were enrolled. Patients underwent both CT and MR imaging within 4 weeks before surgery. In each patient, CT scans were interpreted independently by two radiologists and MR images by two other radiologists at the enrolling institution. The CT and MR images were then interpreted together by two of those radiologists and subsequently reread at the other institutions. Imaging and histopathologic findings were compared and were supplemented when needed with surgical findings. Receiver operating characteristic curve analysis and descriptive statistical analysis were performed. RESULTS: Cases were analyzable in 316 patients: 183 had primary bone tumors; 133 had primary soft-tissue tumors. There was no statistically significant difference between CT and MR imaging in determining tumor involvement of muscle, bone, joints, or neurovascular structures. The combined interpretation of CT and MR images did not statistically significantly improve accuracy. Interreader variability was similar for both modalities. CONCLUSION: CT and MR imaging are equally accurate in the local staging of malignant bone and soft-tissue neoplasms in the specific anatomic sites studied.
Adolescent↗
Primary clear cell sarcoma of bone: a unique site of origin.
Clear cell sarcoma is a rare soft tissue neoplasm, accounting for less than 1% of soft tissue sarcomas. We are presenting a case of a clear cell sarcoma of bone which, to our - knowledge, is the only report of a , primary clear cell sarcoma of bone.
Adolescent↗
Reproducibility of MDM2 and CDK4 staining in soft tissue tumors.
MDM2 and CDK4 immunostaining can be useful adjuncts in diagnosing liposarcoma among soft tissue neoplasms. We examined the reproducibility of MDM2 and CDK4 staining between 2 laboratories and between tissue microarrays and whole tissue sections. Sixty-two soft tissue tumors were immunostained at the Bergonié Institute, Bordeaux, France, and the Curie Institute, Paris, France. We also examined 203 soft tissue neoplasms on standard tissue sections and tissue microarrays. There was high concordance of results obtained from the 2 laboratories (with 2 different pathologists) for MDM2 (kappa, 0.93) and CDK4 (kappa, 0.8) staining. There also was excellent concordance between results on tissue microarray and on whole tissue sections for MDM2 (kappa, 0.80) and CDK4 (kappa, 0.93). Immunostaining for MDM2 and CDK4 is a reproducible technique that may be exported to different laboratories for routine use. Tissue microarray is indicated for studying large series.
Biomarkers, Tumor↗
MR diagnosis of superficial femoral artery aneurysm, primarily misdiagnosed as soft tissue tumor. A case report.
We report a 84-year-old man with an aneurysm of the superficial femory artery (SFA). Primarily, after B-mode sonography soft tissue neoplasm was suspected and sectional MR imaging was performed for further diagnostics. Because of blood flow related ghost artifacts, sectional MR led to the diagnosis of an partially thrombosed aneurysm without angiography. Initially, the differential diagnosis of an SFA aneurysm, which is extremely rare, has not been considered. Soft tissue neoplasms may have a similar appearance to partially thrombosed aneurysms. This case demonstrate the importance to notice also artifacts in sectional MRI which can give important informations for correct diagnosis.
Aged↗
Peripheral neurogenic tumors of the soft tissues in children and adolescents: a clinicopathologic study of 139 cases.
A review of over 900 soft tissue neoplasms in children and adolescents revealed 139 neurogenic tumors in 122 patients from newborn to 20 years of age, which had been accessioned in a 25-year period. Based upon clinicopathologic criteria, 84 (60%) tumors were regarded as unequivocally benign or borderline and the remaining 55 (40%) were malignant. The average age at diagnosis for the entire series was 10 years; the sex distribution was approximately equal; and the trunk (66, 48%) was the most frequent topographic site, followed by the head and neck (39, 29%) and extremities (31, 23%). Eighteen tumors (13%) were recognized at birth. Twenty-one patients (17%) had the stigmata of von Recklinghausen's neurofibromatosis (VRN). Neurofibromas and cellular peripheral nerve sheath tumors accounted for 43% (60 cases) of the diagnoses; these 60 neoplasms occurred in 41 patients, 12 of whom had VRN. The second largest category of neoplasms (38 cases, 28%) was the primitive neuroectodermal tumor (PNET) including the subset of "malignant small cell tumor of thoracopulmonary origin" or Askin tumor. The PNETs other than the Askin tumor (26 cases) presented mainly on the trunk (63%) and head and neck region (33%), showed a male predilection (58%) and had a mean age at diagnosis of 7 years. Askin tumors (12 cases) were, by definition, confined to the chest wall or thoracic cavity, showed a female predilection (71%), and presented in the second decade (82%, mean age 14 years). There were 16 (11%) malignant peripheral nerve sheath tumors, seven of which occurred in children with VRN. Six other categories of neurogenic tumors constituted the remaining cases. Our findings indicate that neurogenic neoplasms are an important nosologic group of soft tissue tumors in the pediatric population.
Adolescent↗
Demonstration of a malignant soft-tissue lymphoma during triple-phase skeletal scintigraphy.
Malignant soft-tissue neoplasms may be demonstrated in the course of triple-phase skeletal scintigraphy due to the presence of either abnormal vascularity or calcifications. We report the unusual case of a malignant lymphoma which was detected in the vascular phases of a triple-phase skeletal scintigraphic study but was not visualized utilizing conventional radioangiographic techniques. Lymphoma should be included in the differential diagnosis of soft-tissue neoplasms demonstrable by scintiangiography.
Edema↗
Vulvar soft tissue tumors.
OBJECTIVE: To report our incidence of soft tissue tumors at this site and to discuss various prognostic factors. METHODS: All patients with a diagnosis of vulvar soft tissue neoplasms were studied from a prospective database at the Royal Marsden Hospital between January 1985 and July 2001. RESULTS: Seventeen vulvar soft tissue neoplasms (11 malignant and six benign) were treated during this period. Leiomyosarcoma (n = 5) and aggressive angiomyxoma (n = 4) were the most frequent histologic types. According to the grade, there were four G3, three G2, three G1 and could not be assessed in one patient. Local recurrence occurred in six patients with sarcoma (three with high grade and one each with intermediate, low, and undetermined grade). In this group, five patients had negative microscopic margins and one patient had positive microscopic margins on excision. All three women with low-grade sarcomas are alive without evidence of disease. Three patients with aggressive angiomyxoma also had a local recurrence. CONCLUSION: Surgical excision is the primary treatment where possible. The grade of the tumor is an important predictor for local recurrence and outcome. Aggressive angiomyxoma is a local problem. Leiomyosarcoma and aggressive angiomyxoma are the most frequent histologic types.
Adult↗