Synovial sarcoma is a rare tumour found in soft tissue; it is a mesenchymal spindle cell tumour that is not related to the synovial membrane. This tumour has a low incidence, and the most frequent place of occurrence is the lower extremities in young adults. Synovial sarcoma of the head and neck accounts for 3%-5% of sarcomas in this anatomical region. The tumor in the nasal cavity is less than 1%. The treatment of choice for synovial sarcoma of the head and neck is complete surgical excision of the tumour mass followed by adjuvant radiotherapy.
Humans ; Nasal Cavity ; pathology ; Nose Neoplasms ; diagnosis ; radiotherapy ; surgery ; Paranasal Sinuses ; pathology ; Radiotherapy, Adjuvant ; Sarcoma, Synovial ; diagnosis ; radiotherapy ; surgery

Chromosomal Proteins, Non-Histone ; metabolism ; DNA-Binding Proteins ; metabolism ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Keratins ; metabolism ; Male ; Mucin-1 ; metabolism ; Phosphopyruvate Hydratase ; metabolism ; Rhabdoid Tumor ; metabolism ; pathology ; surgery ; Rhabdomyosarcoma ; metabolism ; pathology ; S100 Proteins ; metabolism ; SMARCB1 Protein ; Sarcoma ; metabolism ; pathology ; Sarcoma, Clear Cell ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Transcription Factors ; metabolism ; Vimentin ; metabolism

Adolescent ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leg ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Sarcoma, Clear Cell ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery

Actins ; metabolism ; Calcium-Binding Proteins ; metabolism ; Calmodulin-Binding Proteins ; metabolism ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Leiomyomatosis ; metabolism ; pathology ; surgery ; Leiomyosarcoma ; pathology ; Microfilament Proteins ; metabolism ; Middle Aged ; Receptors, Estrogen ; metabolism ; Receptors, Progesterone ; metabolism ; Rhabdoid Tumor ; metabolism ; pathology ; surgery ; Sarcoma, Endometrial Stromal ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology ; surgery ; Vascular Neoplasms ; metabolism ; pathology ; surgery ; Veins ; pathology ; Vimentin ; metabolism

Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm that shows benign histologic features but may have clinical course of malignant disease. It has been reported to occur in the thigh, inguinal area, axilla, shoulder, neck, perineum or buttock. However, there have been few cases of abdominal organ involvement. A 21-year-old woman presented with a large palpable abdominal mass. A 7x4 cm sized round soft tissue tumor at right upper quadrant area was identified by abdominopelvic CT scan. Percutaneous ultrasound-guided biopsy revealed features of spindle cell tumor. On exploration, the tumor originated from transvers colon and was attached to gastrocolic ligament, transverse mesocolon and stomach. The tumor could be dissected with transverse colectomy and partial gastrectomy. The excised tumor, measuring 7x5x5 cm, was well demarcated and appeared as an ovoid mass with firm and myxoid cut surface. She was diagnosed with low-grade fibromyxoid sarcoma arising from transverse colon, and is currently being followed-up without recurrence or metastasis.
Colon, Transverse ; Female ; Humans ; S100 Proteins/metabolism ; Sarcoma/*diagnosis/pathology/surgery ; Soft Tissue Neoplasms/*diagnosis/pathology/surgery ; Stomach Neoplasms/pathology/secondary ; Tomography, X-Ray Computed ; Young Adult

Adult ; Angiomyolipoma ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangiopericytoma ; pathology ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Nephrectomy ; Sarcoma, Synovial ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Chondroblastoma ; pathology ; Chondroma ; pathology ; Chondrosarcoma ; metabolism ; pathology ; surgery ; Cricoid Cartilage ; Diagnosis, Differential ; Humans ; Laryngeal Neoplasms ; metabolism ; pathology ; surgery ; Laryngectomy ; Lymph Node Excision ; Male ; Middle Aged ; Osteoblastoma ; pathology ; Osteosarcoma ; pathology ; S100 Proteins ; metabolism ; Sarcoma, Clear Cell ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Adenoma ; metabolism ; pathology ; Adult ; Alkaline Phosphatase ; metabolism ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Diagnosis, Differential ; Ear Canal ; Ear Neoplasms ; metabolism ; pathology ; radiotherapy ; surgery ; GPI-Linked Proteins ; metabolism ; Humans ; Isoenzymes ; metabolism ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Mast-Cell Sarcoma ; metabolism ; pathology ; Proto-Oncogene Proteins c-kit ; metabolism ; Seminoma ; metabolism ; pathology ; radiotherapy ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicopathologic features, immunophenotype, molecular genetics and differential diagnosis of solid variant of angiomatoid fibrous histocytoma.

METHODSThe clinicopathologic features of 3 cases of solid variant of angiomatoid fibrous histocytoma were analyzed and the literature was reviewed.

RESULTSThere were a total of 2 males and 1 female. The age of patients ranged from 9 to 12 years. The patients presented with a painless mass located in left forearm, left knee or back. The lesions were treated by complete surgical resection. On gross examination, the tumors varied from 1.6 cm to 4.5 cm in greatest dimension. They were well-circumscribed and had pale yellow to grayish-red solid cut surface. Histologically, the tumor was composed of histocytoid cells arranged in sheet-like pattern. A fibrous pseudocapsule surrounded by lymphocytes and plasma cells was identified. Immunohistochemical study showed that the tumor cells in all cases were positive for vimentin and CD68. They were negative for S100 protein, cytokeratin, CD34, CD31, smooth muscle actin, CD35, CD21 and CD30. Two cases also expressed CD99 and one of them was positive for desmin and epithelial membrane antigen. Fluorescence in-situ hybridization was positive for EWSR1 gene.

CONCLUSIONSSolid type represents a variant of angiomatoid fibrous histocytoma and is considered as tumor of borderline malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry and EWSR1 gene study are helpful in further delineation and differential diagnosis. Complete resection or wide local excision with post-operative follow up is the main modality of treatment.

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Back ; Calmodulin-Binding Proteins ; genetics ; Child ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Forearm ; Histiocytoma, Malignant Fibrous ; genetics ; metabolism ; pathology ; surgery ; Humans ; Knee ; Male ; Neoplasms, Muscle Tissue ; pathology ; Neurilemmoma ; metabolism ; pathology ; RNA-Binding Protein EWS ; RNA-Binding Proteins ; genetics ; Soft Tissue Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Actins ; metabolism ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; pathology ; Humans ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism