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Immunohistochemical analysis of soft tissue sarcomas. Comparisons with electron microscopy.

Soft tissue sarcomas are capable of causing diagnostic bewilderment for the surgical pathologist, because of their diversity of differentiation and overlapping histologic appearances. Immunohistochemical studies provide a means whereby this confusion can be resolved in many cases, and electron microscopy also may play a useful role in this process. This overview presents a summary of results of these 2 adjuvant modalities of pathologic analysis, as applied to the spectrum of malignant soft tissue neoplasms.

Humans↗

Recognizable magnetic resonance imaging characteristics of intramuscular myxoma.

Extremity intramuscular myxoma is an uncommon, deep, benign soft-tissue neoplasm. It is characterized clinically by slow growth and minimal symptoms. Resection achieving wide surgical margins is curative. This article presents the imaging characteristics of five extremity intramuscular myxomas. Images of this benign neoplasm, especially magnetic resonance imaging, are characteristic enough for preoperative recognition. This knowledge can facilitate treatment by avoiding biopsy, permitting primary myectomy, and assisting the pathologist in diagnosing a neoplasm that frequently has overlapping histologic characteristics with other soft-tissue tumors.

Adult↗

Nuchal fibroma: a clinicopathological review.

Nuchal fibroma, or collagenosis nuchae, is a benign soft tissue tumor that arises from the posterior cervical subcutaneous tissue, with a predilection for the interscapular and paraspinal regions. Because of its benign clinical course and its close histopathologic similarity to other benign head and neck lesions, this lesion may be misdiagnosed and underreported. The purpose of this paper is to review the histopathologic and radiologic findings unique to nuchal fibroma, and compare and contrast it to the other soft tissue neoplasms within the clinical differential diagnosis. These include several benign (elastofibroma, lipoma, fibrolipoma, nodular fasciitis) and rare malignant entities (fibrosarcoma, liposarcoma, fibromatosis).

Adult↗

Bilateral metastatic renal synovial sarcoma.

Synovial sarcoma is a malignant soft-tissue neoplasm, usually arising in close association with the joints and generally carrying a poor prognosis. We describe the first report of bilateral renal metastases from synovial sarcoma in a long-term survivor. Treatment consisted of systemic therapy with bilateral partial nephrectomies. The patient was disease free at 1 year of follow-up.

Amputation, Surgical↗

Leiomyosarcoma: a rare pedal finding.

Leiomyosarcoma of the skin and subcutaneous tissue is a rare soft tissue neoplasm comprised of spindle-shaped smooth muscle cells. The statistical incidence of superficial leiomyosarcomas varies from 2.3% to 5.3% of malignant soft tissue tumors and from 4.0% to 6.5% of soft tissue sarcomas. Its occurrence in the foot is even more unusual. In a literature review extending from 1936-present, the authors have been able to locate only 12 known cases of leiomyosarcoma involving the foot and ankle. A presentation of the incidence, demographics of distribution, clinical characteristics, histopathologic features, prognosis, and treatment of leiomyosarcoma will be presented along with a unique case history involving a recalcitrant heel ulcer complicated by leiomyosarcoma.

Aged↗

Alveolar soft part sarcoma in brain with cardiac metastasis: a case report.

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The coexistence of ASPS with cardiac metastasis is quite rare, in particular. In general, the sarcoma is a malignant disease and grows very fast. However, the mean survival time of patients with ASPS is relatively long. Patients who are diagnosed with localized disease usually have a favorable prognosis, while those who present with widespread metastases usually have a poor prognosis and ultimately, succumb to their disease. The use of modern treatment modalities, such as combination of surgery, and radiotherapy, may significantly prolong the survival time in many patients. Because of the long-term period with symptomatic brain tumors in this disease, the patients should be treated even in the presence of multiple metastases in other organs.

Adult↗

Spontaneous pelvic hematoma simulating neoplasm: case report and literature review.

Spontaneous hematomas are rare and can present with acute or chronic symptoms. Our patient presented with deep vein thrombosis of the lower extremity associated with a spontaneous pelvic hematoma. This lesion was radiologically and clinically indistinguishable from a soft-tissue neoplasm. The case of a spontaneous pelvic neoplasm in an otherwise healthy young man is presented and the literature reviewed regarding issues of differential diagnosis and clinical management.

Adolescent↗

Clear cell sarcoma with melanin pigment.

A patient with a clear cell sarcoma of tendons and aponeuroses arising in the left sacral area is reported. The tumor grossly contained two black foci that had pigment with the staining characteristics of melanin on light microscopy. Electron microscopy of these areas showed the pigment to be within melanosomes. This is the second report of a neoplasm diagnosed as clear cell sarcoma in which melanin was demonstrated; the possibility that the tumor represents a soft tissue variant of malignant melanoma is discussed. It is suggested that clear cell sarcoma is a heterogeneous entity, and that a number of different soft tissue neoplasms may present with a clear cell pattern, making diagnosis and classification difficult.

Adult↗

Ultrasound of soft tissue abnormalities of the extremities.

Ultrasound may be the preferred initial diagnostic modality for the painful swollen extremity, the palpable extremity mass, and the injured extremity. The role of ultrasound in extremity soft tissue abnormalities, including muscle hemorrhage and injury, inflammatory processes, and soft tissue neoplasms, is the subject of this review.

Abscess↗

[Radioisotopic exploration of sarcomas of the bone and soft tissue].

Radioisotopic methods are widely applied to investigations of bone sarcoma and soft tissue neoplasms. We have at our disposal molecules with osseous, tumoral or vascular tropism. Their use, as single agents or combination, is helpful in positive and differential diagnosis and provides nosological informations. They are also useful in treatment monitoring and in long-term follow-up.

Bone Neoplasms↗

Rhabdoid tumors of soft tissues: a clinicopathologic study of 26 cases enrolled on the Intergroup Rhabdomyosarcoma Study.

Twenty-six cases of malignant soft tissue tumors with features similar to renal rhabdoid tumors were identified among approximately 3,000 childhood sarcomas entered on Intergroup Rhabdomyosarcoma Studies I-III. The tumors consisted of polygonal cells with vesicular nuclei and prominent nucleoli and cytoplasmic intermediate filament inclusions as identified by electron microscopy and immunohistochemistry. The growth pattern was predominantly solid or solid-trabecular. Immunohistochemistry showed vimentin, wide spectrum keratin, and epithelial membrane antigen to be the most consistent antigenic phenotypes. Eleven patients were infants less than 1 year of age. The tumors affected predominantly soft tissues of proximal extremities, trunk, and retroperitoneum/pelvis/abdomen. Nineteen patients died within 1 to 82 months (median, 6 months) from the start of treatment. Five patients have survived the disease for 2 to 13 years. When compared with the survival analysis of 991 Intergroup Rhabdomyosarcoma Study II patients, it was obvious that this group of tumors fares very poorly (P less than .001). The tumor belongs to the group of soft tissue neoplasms showing mesenchymal and subtle epithelial differentiation, similar to epithelioid sarcoma. Because of its identifiable histology, site and age distribution, and poor outcome, it warrants a status as an independent entity.

Adolescent↗

Dendritic fibromyxolipoma: clinicopathologic study of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma.

Twelve cases are described of a distinctive benign soft tissue lesion that may be mistaken for a sarcoma. The tumors occurred in 11 men and a woman aged 33 to 81 years (mean, 64 years), and measured from 2 to 11 cm in greatest diameter (mean, 6 cm). They were grossly described as soft, well-circumscribed, yellow-gray, with a mucoid cut surface. All cases were superficially located in the subcutis or muscular fascia of the head and neck region or the chest and back. Histologically, the tumors were characterized by a proliferation of spindle or stellate fibroblastic cells variably admixed with mature adipose tissue embedded in an abundant myxoid and collagenized stroma. The spindle and stellate fibroblastic cells were characterized by slender dendritic prolongations of their cytoplasm, which appeared to extend for short distances along connective tissue planes. Electron microscopy in two cases confirmed the dendritic nature of the fibroblastic cells, which showed elongated cytoplasmic processes lacking external lamina and displaying foci of pinocytotic activity. Immunohistochemical studies in 11 cases showed strong positivity of the spindle cells with vimentin, CD34 and bcl-2, and negative staining for smooth muscle actin, muscle-specific actin (HHF35), desmin, S-100 protein, keratin, and EMA. Because of their prominent myxoid stroma and relatively large size, some of these tumors were initially misinterpreted as low-grade sarcomas. Clinical follow-up in five cases, however, showed that the patients were alive and well without evidence of recurrence between 5 and 13 years (mean follow-up, 8 years) after simple local excision. The present cases appear to represent a distinctive form of benign soft tissue neoplasm that should be distinguished from myxoid liposarcoma and other benign and malignant myxoid tumors of superficial soft tissues.

Adult↗

Paravertebral infection (phlegmon) demonstrated by FDG dual-head coincidence imaging in a patient with multiple malignancies.

A 66-year-old woman was referred for a bone scan to assess back pain on a background of breast cancer, melanoma, and rheumatic heart disease. The scan appearance was suspicious for a localized soft tissue neoplasm. An FDG coincidence positron emission tomography (PET) study demonstrated a large FDG-avid soft tissue abnormality. Staphylococcus aureus was isolated from a subsequent needle biopsy. This case illustrates the use of FDG-PET in infection imaging, as well as demonstrating the potential pitfalls in nuclear oncology. Because FDG is not tumor-specific, accumulation in benign lesions may give rise to false-positive results despite a high pretest probability for malignancy.

Aged↗

Pleomorphic fibroma of tendon sheath.

We report an example of fibroma of tendon sheath with pleomorphic bizarre cells without mitoses. The tumor appears to be morphologically identical to a similar tumor in the skin and represents yet another example of a benign soft-tissue neoplasm with disturbing morphological features.

Fibroma↗

[Biometric studies in the cytological diagnosis of soft tissue tumors].

Introducing of quantitative signs (the number of cells on the given area, size of cells, nuclei, optic and spectral characteristics) makes the cytologic diagnosis much more objective. Karyometric studies conducted by the author (the determination of nuclei volume) on cytological smears, obtained from 16 patients with soft tissue tumors, indicated some difference in nuclei size of various histotypes. The studies have evidenced that the method of karyometry associated with cytophotometry will offer the pre-conditions for using karyometry in the practice of pathocytologists and facilitate the finding of algorithms for computing analysis of soft tissue neoplasms.

Cell Nucleus↗

Myxoma of the renal capsule.

Myxomas are uncommon soft-tissue neoplasms, which are extremely rare in the kidney, with only five cases documented in the intraparenchymal location. However, renal capsular myxoma has not yet been reported. We describe a unique case of a clinically detected renal myxoma arising in the capsule. A 37-year-old man receiving treatment for epididymitis sought medical assistance for infertility. A radiological examination incidentally discovered a right renal tumor. The mass intruded into the perirenal tissue and measured 6 cm in major diameter. The resected kidney contained a well-circumscribed gelatinous capsular tumor. It was composed of sparse, bland, slender, spindle-shaped cells scattered in large amounts of basophilic interstitial mucoid material. The tumor cells showed diffuse immunoreactivity for vimentin. Occasional cells stained for alpha-smooth muscle actin and calponin. Reactivity was negative for S100 protein, epithelial membrane antigen, pancytokeratin, neurofilament protein, and h-caldesmon antibodies. Ultrastructural examination revealed fibroblast-like cells with long thin cytoplasmic processes, prominent rough endoplasmic reticulum, a well-developed Golgi complex, and secretory vesicles. No basal lamina was identified around the tumor cells. The differential diagnosis includes many other benign and malignant soft-tissue lesions exhibiting prominent secondary myxoid features. It is important to consider a renal capsular myxoma when examining lesions at this anatomic site to avoid misdiagnoses and to ensure that the patient receives appropriate treatment and prognostic information.

Adult↗

Clear cell sarcoma of soft tissues in children and young adults: the St. Jude Children's Research Hospital experience.

Clear cell sarcoma is a rare soft tissue neoplasm whose clinical behavior and outcome has not been previously characterized. This study reviewed the clinical characteristics and outcome of all children with clear cell sarcoma of the soft tissues who were treated at St. Jude Children's Research Hospital from March 1962 through August 1998. Of 225 children with nonrhabdomyosarcomatous soft tissue sarcomas, 5 (2.2%) were diagnosed with clear cell sarcoma. Median age at diagnosis was 15 years 3 months. Primary sites included the extremities (n = 3), chest wall (n = 1), and abdomen (n = 1). At diagnosis 3 patients had localized disease. Following surgical resection (n = 3), radiotherapy (n = 2), and chemotherapy (n = 1) all three survive disease-free 10, 11, and 90 months after diagnosis, respectively. The remaining two patients with metastatic disease at diagnosis died 21 days and 9 months after diagnosis. Clear cell sarcoma of the soft tissues is rare in pediatrics. Complete surgical resection with negative margins is the most effective treatment for this disease. Patients with metastatic disease are candidates for multiinstitutional chemotherapy trials.

Adolescent↗

[Differential diagnosis of nodular fasciitis].

A clinico-morphological analysis of 32 observations of nodular fascitis enabled the recognition of a number of signs (a rapid growth, polymorphism of the cellular and structural content, infiltration of the adjacent tissues), which might simulate a malignant character of the lesion. Whereas the analysis also revealed some distinguishing features of soft tissue neoplasms resembling nodular fascitis.

Adolescent↗