Soft tissue sarcomas at a glance: clinical, histological, and MR imaging features of malignant extremity soft tissue tumors

Soft tissue sarcomas comprise approximately 1% of malignant tumors. There are more than 50 subtypes, but pleomorphic sarcoma, liposarcoma, leiomyosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor account for 75%. Differentiation between these subtypes is difficult because they often present with a painless enlarging mass, and share many histological and MR imaging features. Nonetheless, subdifferentiation is important because the different subtypes have different prognoses and therapeutic strategies. In this manuscript we discuss the clinical, histological, and MR imaging features of soft tissue sarcomas according to the WHO classification. An overview is provided and differentiating features are discussed that can help to narrow down the differential diagnosis

Highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis

<p>Abstract</p> <p>Purpose</p> <p>To evaluate the characteristics of highly malignant soft tissue sarcoma of the extremity with a delayed diagnosis.</p> <p>Materials and methods</p> <p>The clinical and radiological characteristics of 18 cases of highly malignant soft tissue sarcomas of the extremity with a delayed diagnosis were determined.</p> <p>Results</p> <p>Ten men and eight women of mean age 44.8 years (range, 15-79 years) were included in this study. Seven cases of synovial sarcoma, three cases each of alveolar soft part sarcoma and malignant fibrous histiocytoma, two cases each of highly malignant leiomyosarcoma and myxofibrosarcoma, and one case of clear cell sarcoma were enrolled. Times from tumor detection to diagnosis ranged from 1 to 3 years in most cases; three of the seven synovial sarcoma cases took more than 10 years to diagnose. Of the seven cases of synovial sarcoma, five cases of small, superficial located masses were simply excised without a pre-surgical biopsy. Three cases of alveolar soft part sarcoma showed characteristic T1- and T2-weighted high signal intensities with signal voids in MR images. In addition, one synovial sarcoma patient and one alveolar soft part sarcoma patient showed evidence of calcification on plain radiographs. However, no general characteristic clinical findings were found to be common to the 18 cases.</p> <p>Conclusions</p> <p>Contrary to general expectations, some soft tissue tumors that grow slowly are painless, and those that occur in superficial limbs may be highly malignant. Thus, even when a slow growing, painless superficial mass is encountered in a limb, physicians should keep the possibility of highly malignant soft tissue sarcoma in mind.</p

Alveolar soft part sarcoma: the new primary intracranial malignancy. A case report and review of the literature

The purpose of this paper is to serve as a reference to aid in the management of this poorly understood intracranial malignancy. The authors report their experience treating the eighth ostensible case of a primary intracranial alveolar soft part sarcoma (ASPS). A 21-year-old man presented to hospital after collapsing. He gave a 1-year history of headache, a 2-month history of reduced visual acuity and on examination had left facial paraesthesia with left-sided incoordination. MRI of the brain revealed a large left posterior fossa mass. The patient underwent resection of the tumour with good recovery in function. Immunohistochemical analysis of the tumour specimen confirmed an ASPS, and multimodal imaging in search of an extra-cranial disease primary was negative. A review of the literature yielded only seven other cases of primary intracranial ASPS. A variety of diagnostic imaging modalities were employed in search of a disease primary, as were various combinations of surgical resection, chemotherapy and radiotherapy as treatment. Half of the cases documented delayed disease recurrence. The authors discuss the following: the unique radiological and immunohistological characteristics of this disease including the potential for its misdiagnosis; the investigations required to diagnose a primary intracranial ASPS; the efficacy of current medical and surgical treatment options and the factors that will aid in prognostication. This is the first review of this new primary intracranial malignancy. From our analysis, we offer a joint radiological and immunohistochemical algorithm for the diagnosis of primary intracranial ASPS and specific operative considerations prior to resection

Alveolar soft-part sarcoma of the retroperitoneum

The definitive version is available at www.blackwell-synergy.com.ArticleInternational Journal of Urology. 13(10): 1355-1357 (2006)journal articl

Uso exitoso de la radioterapia conformada tridimensional como tratamiento adyuvante del sarcoma alveolar de partes blandas. Reporte de un caso

Introduction: Alveolar soft part sarcoma is a very rare and aggressive type of sarcoma. Although its histology and genetic characteristics have been identified, the benefits of adjuvant radiotherapy for its treatment are still being studied. Case presentation: In November 2007, a 21-year-old woman presented with a primary tumor in the right thigh, with histological and immunohistochemical confirmation of an alveolar soft part sarcoma, which was totally resected in December 2007. Also, the large size of the mass suggested an unfavorable evolution. Two years after the first surgery, two metastatic tumors were detected in the right lung, which were completely resected separately. Two years later, the patient had two independent relapse events, five months apart: a mass in the right tight, and a metastatic tumor in the adrenal gland together with relapse in the tight. All tumors were successfully resected. In June 2014, after the last local relapse, adjuvant radiotherapy was started because of the risk of thigh amputation. At the end of treatment, the patient’s general condition was good. Currently, at age 34, the patient is monitored through periodic evaluations, showing disease regression and stabilization. Conclusions: Currently, it is known that radiation not only produces cytotoxic effects on the target region, but also induces an immune system-mediated systemic response with potential antimetastatic properties. The emerging radiobiological paradigms should be considered, particularly since they could explain some encouraging and unexpected results such as those described in this case.Introducción. El sarcoma alveolar de partes blandas es un raro y agresivo tipo de sarcoma. Aunque se han identificado sus características histológicas y genéticas, todavía se están estudiando los beneficios de la radioterapia adyuvante en su tratamiento. Presentación del caso. En noviembre de 2007, una mujer de 21 años se presentó con un tumor primario en el muslo derecho, con confirmación histológica e inmunohistoquímica de sarcoma alveolar de partes blandas y que fue completamente removido en diciembre de 2007. La masa mostró un gran tamaño, sugiriendo una evolución desfavorable. Dos años después de la primera cirugía, se detectaron dos tumores metastásicos en el pulmón derecho, que también fueron removidos, de forma separada. Dos años después, la paciente tuvo dos relapsos, separados por cinco meses: una masa en el muslo derecho, y un tumor metastásico en la glándula suprarrenal junto con una recaída en el muslo. Todos los tumores fueron extirpados con éxito. En junio de 2014, después de la última recaída local, el muslo estaba en riesgo de ser amputado, por lo que se decidió iniciar radioterapia adyuvante. Al final del tratamiento, la condición general de la paciente fue buena. Actualmente, ya con 34 años, es evaluada periódicamente, mostrando regresión y estabilización de la enfermedad. Conclusiones. Actualmente, se sabe que la radiación no solo produce efectos citotóxicos en la región objetivo, sino que también induce una respuesta sistémica mediada por el sistema inmune, con propiedades potencialmente antimetastásicas. En este sentido, se sugiere considerar los paradigmas radiobiológicos emergentes, ya que estos podrían explicar algunos resultados alentadores e inesperados como los descritos en este caso.Fil: Ocolotobiche, Eliana Evelina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico CONICET- La Plata. Instituto de Genética Veterinaria "Ing. Fernando Noel Dulout". Universidad Nacional de La Plata. Facultad de Ciencias Veterinarias. Instituto de Genética Veterinaria; Argentina. Universidad Nacional de La Plata. Facultad de Ciencias Exactas; Argentina. Red CIO-Terapia Radiante S.A.; ArgentinaFil: Pérez Duhalde, Esteban. Red CIO-Terapia Radiante S.A.; Argentina. Universidad Nacional de La Plata. Facultad de Ciencias Médicas; ArgentinaFil: Güerci, Alba Mabel. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico CONICET- La Plata. Instituto de Genética Veterinaria "Ing. Fernando Noel Dulout". Universidad Nacional de La Plata. Facultad de Ciencias Veterinarias. Instituto de Genética Veterinaria; Argentina. Universidad Nacional de La Plata. Facultad de Ciencias Exactas; Argentina. Red CIO-Terapia Radiante S.A.; Argentin

Alveolar soft part sarcoma masquerading as lipoma: a case report

We report herein, a rare case of a 14-year-old child with alveolar soft part sarcoma. The patient initially noticed a progressively growing mass over medial side of right distal thigh. Although clinically the swelling appeared to be lipoma, the needle biopsy confirmed it to be alveolar soft part sarcoma. Patient was managed by wide excision, followed by adjuvant chemotherapy

Metastasis to the gluteus maximus muscle from renal cell carcinoma with special emphasis on MRI features

<p>Abstract</p> <p>Background</p> <p>The skeletal muscle is an unusual site for metastasis from renal cell carcinoma (RCC). Metastatic RCC must be differentiated from benign primary soft-tissue tumors because aggressive surgical resection is necessary.</p> <p>Case presentation</p> <p>We present the case of a 65-year-old man with metastatic RCC in the gluteus maximus muscle (3.8 cm in diameter) found on enhanced computed tomography (CT) 6 years after nephrectomy. Retrospectively, the small mass (1 cm in diameter) was overlooked 5 years earlier on enhanced CT. Because the growth of the lesion was slow, benign tumor was a differential diagnosis. However, magnetic resonance imaging (MRI) showed that the mass had high-signal intensity on T1- and T2-weighted images (WIs) compared to that of skeletal muscle, with mild enhancement by Gadolinium. The MRI features were unusual for most soft-tissue tumors having low-signal intensity on T1-WI and high-signal intensity on T2-WI. Therefore, under a diagnosis of metastatic RCC, the lesion was resected together with the surrounding skeletal muscle. The histology was confirmed to be metastatic RCC.</p> <p>Conclusion</p> <p>MRI features of metastatic RCC may be beneficial in differentiating it from primary soft-tissue tumor.</p

Imaging findings in noncraniofacial childhood rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood. This paper is focuses on imaging for diagnosis, staging, and follow-up of noncraniofacial RMS

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