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PubMed1994

Sarcoma metastatic to the brain: results of surgical treatment.

We report on 21 patients surgically treated for intraparenchymal brain metastasis from sarcoma, including six osteosarcomas, four leiomyosarcomas, three malignant fibrous histiocytomas, two alveolar soft-part sarcomas, two Ewing's bone sarcomas, one extraskeletal osteosarcoma, one extraskeletal Ewing's sarcoma, and two unclassified sarcomas. Median survival after craniotomy was 11.8 months. Patients with a preoperative Karnofsky performance score of > 70 survived for 15.7 versus 6.6 months for those with a Karnofsky performance score < or = 70. Patients. undergoing complete resection survived 14.0 versus 6.2 months for patients undergoing incomplete resection. Patients with evidence of lung metastases at the time of surgery survived 11.8 months, which was similar to the 10.5-month survival for patients with disease limited to the brain. The two patients with alveolar soft-part sarcoma are alive at 16 and 25 months after surgery. We conclude that surgery is effective in treating selected patients with sarcoma metastatic to the brain and that patients with metastasis from alveolar soft-part sarcoma may have a relatively good prognosis if they are surgically treated. The complete removal of all brain metastases and a Karnofsky performance score > 70 are associated with a favorable prognosis, whereas the presence of concurrent lung metastases is not a contraindication to surgery.

Adolescent↗
PubMed1998

Sarcoma metastatic to the brain: a series of 15 cases.

METHODS: We report on 15 patients surgically treated for intraparenchymal brain metastases from sarcoma, including six osteosarcomas, five leiomyosarcomas, two malignant fibrous histiocytomas, and two alveolar soft-part sarcomas (ASPS). RESULT: Median survival after craniotomy was 9.3 months. Patients with a preoperative Karnofsky performance score of > 70 survived for 12.8 versus 5.3 months for those with a Karnofsky performance score < 70 (p=0.03). Patients with evidence of only lung metastases at the time of surgery (nine cases) survived 8.6 months, which was similar to the 10.4-month survival for patients with disease limited to the brain (p=0.1). The two patients with alveolar soft-part sarcomas are alive at 15 and 20 months after surgery. CONCLUSION: We conclude that surgery is effective in treating selected patients with sarcoma metastatic to the brain and that patients with metastasis from ASPS may have a relatively good prognosis if they are surgically treated. The complete removal of all brain metastases and a Karnofsky performance score > 70 are associated with a favorable prognosis; the presence of concurrent lung metastases is not a contraindication to surgery.

Adolescent↗
PubMed2000

Membrane-bound paracrystalloid structures in alveolar rhabdomyosarcoma.

We report here a case of an alveolar rhabdomyosarcoma in a 25-year-old woman. Ultrastructural examination revealed a few periodical membrane-bound crystalloids, similar to those regarded as specific for alveolar soft-part sarcoma. Our study provides evidence supporting a myogenic origin of those periodic structures, and simultaneously a myogenic origin of alveolar soft-part sarcoma.

Adult↗
PubMed1988

Sarcoma metastatic to the brain.

Five patients with nonlymphomatous sarcoma metastatic to the brain are presented and the literature reviewed. In 50 patients, brain metastasis was verified pathologically and some case data were available, including the five reported here. In five patients, there was pathologic verification but no case data. Thirty-nine other patients were found without such verification. Reportedly, most types of sarcomas are able to metastasize to the brain, and are represented in these 94 patients. Fibrosarcoma and alveolar soft-part sarcoma tumors metastasize to the brain without regard to treatment technique. There may be a group of tumors, including malignant fibrous histiocytoma, rhabdomyosarcoma, and perhaps leiomyosarcoma and osteosarcoma, in which the incidence of brain metastases has increased with improved sarcoma chemotherapy (CT). In this group particularly, but also in alveolar soft-part sarcoma and others, the presence of lung metastases may increase the probability of brain metastasis occurring subsequently.

Adolescent↗
PubMed1994

Myogenic regulatory protein expression in adult soft tissue sarcomas. A sensitive and specific marker of skeletal muscle differentiation.

Myogenic regulatory protein (MyoD1) is a DNA-binding nuclear protein that initiates myogenesis in mesenchymal stem cells. Its expression has proved a very sensitive marker of myogenic differentiation in malignant tumors of childhood. In this study, the reliability of MyoD1 expression as marker of skeletal muscle differentiation has been tested in 38 cases of adult-type sarcomas. Frozen sections were stained with the monoclonal antibody 5.8A (MAb 5.8A) developed against recombinant wild-type murine MyoD1. Routinely processed formalin-fixed sections from the same cases were reviewed and immunostained with a panel of antibodies commonly used to detect muscle differentiation: myoglobin, fast-myosin, desmin, muscle-specific actin, alpha-smooth muscle actin. Four cases positively stained with MAb 5.8A: three were high-grade spindle cell sarcomas, the fourth case was an alveolar soft-part sarcoma. Only tumors in which skeletal muscle histogenesis was inferred on the basis of histology and/or immunohistochemistry with conventional muscle markers stained positively for MAb 5.8A. All the other tumors tested, including leiomyosarcomas (11), liposarcomas, (10, including four dedifferentiated liposarcomas), fibrosarcomas (three), malignant fibrous histiocytomas (two), synovial sarcomas (two), and malignant peripheral nerve sheath tumors (two), were negative for MAb 5.8A. The high level of sensitivity and specificity of MyoD1 expression indicates the value of this marker in the diagnosis of soft tissue sarcomas.

Adolescent↗
PubMed2002

Ultrastructure of oral sarcoma.

Sarcoma of the oral region is extremely rare and ultrastructural studies of the tumor are limited in number. We collected oral sarcomas, such as fibrosarcoma, malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, rhabdomyosarcoma, angiosarcoma, alveolar soft-part sarcoma, solitary plasmacytoma, and osteosarcoma, and performed ultrastructural studies of these tumors. The value of these studies for an understanding of the biological behavior of the tumors was then investigated. In these studies, electron microscopic examinations of oral sarcoma were of assistance in our attempt to establish correct diagnosis and histogenesis. Data from the studies of oral sarcoma by light microscopy, electron microscopy, and immunohistochemistry should be accumulated.

Fibrosarcoma↗
PubMed2000

Cytogenetic analysis of rare orbital tumors: further evidence for diagnostic implication.

INTRODUCTION. The pathologic diagnosis, and hence the management, of rare tumors of the orbit can represent a challenge. An increasing number of tumors have been shown to display specific chromosomal abnormalities detectable by cytogenetic analysis. MATERIALS AND METHODS. Two examples of rare primary orbital tumors are presented in which the correct pathologic diagnosis was either established or confirmed by cytogenetic analysis. Small pieces of the tumor were obtained at the time of biopsy and placed immediately, in a sterile fashion, in fresh culture medium. The cells were Giemsa stained and processed for chromosome analysis. RESULTS. In the first case, a 12-year-old boy was diagnosed with a sino-orbital mass after sustaining minor trauma. On biopsy, the pathologic examination was at first consistent with a Burkitt-like lymphoma, until cytogenetic analysis showed a t(8;21)(q22;q22) translocation, specific for granulocytic sarcoma, which corresponds to the localized solid form of acute myeloblastic leukemia. Consequently, proper chemotherapy was re-oriented and remission was achieved. In the second case, a 2-year-old boy presented with a well-vascularized orbital tumor. The histopathologic diagnosis of alveolar soft-part sarcoma of the orbit was confirmed by the finding of a specific t(X;17)(p11;q25) translocation. After two unsuccessful courses of chemotherapy, wide excision surgery with primary reconstruction brought remission. CONCLUSIONS. Cytogenetic analysis should be considered in the diagnosis of any orbital tumor which is suspected of malignancy.

Journal Article↗
PubMedDate not supplied

The pathology of head and neck tumors: fibroadipose tissue and skeletal muscle, Part 8.

Benign and malignant tumors originating from mesenchymal cells destined to become lipoblasts and myoblasts affect the head and neck with contrasting frequencies. Lipomas and especially liposarcomas are unusual lesions above the clavicles but when found there behave in a biologic manner identical to that of their counter-parts at other anatomic sites. Myogenic tumors, on the other hand, have a predilection for the head and neck, and for rhabdomyosarcomas this predilection is accentuated in childhood. Combination therapy of rhabdomyosarcomas has obviated radical surgery as a method of treatment, and many sites in the head and neck have benefited prognostically by this treatment. Success, however, is dependent on clinical stage of disease, and rhabdomyosarcomas of the nasopharynx, paranasal sinuses, and middle ear remain more resistant to short-term cures because of the extent of the neoplasm. A review of the clinicopathologic aspects of granular-cell tumors and alveolar soft-part sarcomas is also presented because it has been suggested that these tumors have a myogenous origin.

Adult↗
PubMed1978

[On the occurrence of so-called clear cells in salivary gland tumors. Ultrastructure and differential diagnosis (author's transl)].

Clear cell tumors of the salivary glands are monomorphic clear cell adenomas, clear cell carcinomas, clear cell variants of acinic cell and mucoepidermoid tumours, sebaceous cell tumors, salivary duct carcinomas and pleomorphic adenomas with clear cell sectors. At the light microscopical level the descriptive term of the clear cell comprises cell types of different origin and functional importance which can be differentiated by cytochemistry and electron microscopy. The following cell types were analysed precisely: indifferent duct cells (small formation of organelles, desmosomes), storing striated duct cells (glycogen granules, multiple mitochondrias, basal labyrinth), myoepithelial cells (myofilaments, pinocytosis vesicles, lipofuscin granules, hemidesmosomes), goblet cells (mucous vacuoles, basal endoplasmatic reticulum), sebaceous cells (lipid droplets, microvilli, desmosomes) and clear acinic cells (electron pale secretory granules, small mitochondrias, small golgi apparatus). Clear cell tumor types of the salivary gland region which primarely do not derive from the salivary gland tissue must also be included in the differential diagnoses. These are metastases of hypernephroid renal carcinomas, paragangliomas, glomus tumors of Masson, granular cell tumor and alveolar soft-part sarcomas.

Adenoma↗
PubMed2026

GPNMB-directed CAR T cell therapy against MiT/TFE-family fusion-driven solid tumors.

Chimeric antigen receptor (CAR) T cell therapy for solid tumors is constrained by the scarcity of safe, uniformly expressed cell-surface targets. Here we identify glycoprotein NMB (GPNMB)-an MiT/TFE-family fusion-driven protein-as being highly, homogeneously and stably expressed in primary and relapsed alveolar soft-part sarcoma (ASPS) and translocation renal cell carcinoma. We develop a GPNMB-directed CAR T cell product, GCAR1, which demonstrates potent activity against patient-matched cells, organoids and xenograft models. Post hoc interim analysis of a first-in-human open-label, individual-participant trial ( NCT07104682 ) for a participant with relapsed/refractory, metastatic ASPS showed that GCAR1 induces stable disease for up to 3&#x2009;months, accompanied by resolution of many nontarget lesions (primary endpoint), and is well tolerated. GCAR1 T cells expand in peripheral blood as a polyclonal population and remain detectable for 1&#x2009;month. Spatial transcriptomics identified immunosuppressive niches in a treatment-resistant lesion and immune checkpoint blockade synergized with GCAR1 in a xenograft model. Altogether, our data provide a proof of concept for treating GPNMB-expressing solid tumors with GCAR1 and more broadly targeting surface antigens driven by oncogenic gene fusions with CAR T cell therapies.

Animals↗
PubMed1987

Misdiagnosed localized arteriovenous malformation.

We report a case of an 11-year-old girl with a pulsatile mass in the thigh diagnosed by angiography as localized arteriovenous malformation. Recent increase in tenderness and size of the lesion brought the patient to hospital and excision was performed at the parents' request. Subsequent histologic examination revealed that this lesion was an alveolar soft-part sarcoma in disguise. The angiographic and pathologic results are presented. Other investigators should be aware of this uncommon and highly vascular sarcoma, which might be misdiagnosed as a localized arteriovenous malformation and thus treated only conservatively.

Arteriovenous Malformations↗
PubMed2004

Deep venous thrombosis revealed during ultrasound-guided femoral nerve block.

Ultrasound imaging used to facilitate performance of a femoral nerve block also affords imaging of adjacent anatomical structures. Following a fracture of the femur, an ultrasound guided femoral nerve block (UGFNB) was performed to provide analgesia; this led to the incidental finding of a previously undiagnosed femoral vein thrombosis (DVT), resulting in a change in patient management before surgery. An inferior vena cava (IVC) filter was placed before intramedullary nailing of the fracture.

Adult↗
PubMed2003

Reconstruction of the pelvis after resection of tumors about the acetabulum.

Periacetabular resections for primary malignancies and metastatic disease require reconstruction to restore weightbearing along anatomic axes. Without reconstruction, patients are unable to ambulate independently, and are left with a disfigured pelvis and shortened limb. The current authors describe a reconstruction technique using Steinmann pins augmented with methylmethacrylate reconstruction, and autoclaved autografting, in combination with total hip arthroplasty, after resection of primary sarcomas of the pelvis. For this study, the results of 15 patients at two institutions who had surgery by the same surgeons were retrospectively reviewed. The patients had primary malignant tumors of the pelvis, and had limb-sparing resections between 1985 and 2000. Three measures of outcome were evaluated: survival, function, and pain. The surgical method uses Steinmann pins with bone cement to fill in areas of bone loss that cannot be reconstructed with autoclaved autograft. A constrained polyethylene acetabular component is cemented into this bed. Twelve patients had chondrosarcoma and one patient had osteosarcoma. The remaining two patients had alveolar sarcoma of soft parts. Six patients died of disease. Seven patients were alive with no evidence of disease, and two were alive with disease at the most recent followup. Early return to ambulation without assistive devices occurred in nine of 15 patients. The functional outcome of this technique compares favorably with others reported. Sixty percent of patients ambulated independently without assistive devices, compared with 0% to 10% in other studies reviewed. The time required for independent gait is similar to recovery from a total hip arthroplasty. Moreover, this method minimized leg length discrepancy, while also producing a favorable cosmetic result.

Acetabulum↗
PubMed2003

Highly vascular pelvic tumor causing high-output heart failure because of massive arteriovenous shunting: a case report.

A 53-year-old Japanese woman underwent investigation of her heart murmur. A continuous abdominal bruit was heard. Blood gas analysis and chest X-ray showed congestive heart failure. Enhanced computed tomography of the pelvis showed a 10 x 4 cm hypervascular tumor in the retroperitoneal space. Cardiac catheterization disclosed a cardiac output of 13.2 L/min and a step-up of oxygen at the right common iliac vein. Abdominal aortic angiography showed an extremely vascular pelvic tumor and rapid filling of the inferior vena cava. This is a rare case of a highly vascular pelvic tumor causing high-output heart failure because of th massive arteriovenous shunting.

Arteriovenous Fistula↗