Objective: To investigate the clinicopathological features, immunophenotype, molecular features and differential diagnosis of primary synovial sarcoma of the lung (PSSL). Methods: Twelve cases of PSSL were collected at Henan Provincial People's Hospital, during May 2010 and April 2021, and their clinicopathological parameters were summarized. SS18-SSX, H3K27Me3, and SOX2 were added to the original immunomarkers to evaluate their diagnostic value for PSSL. Results: The age of 12 patients when diagnosed ranged from 32 to 75 years (mean of 50 years). There were 7 males and 5 females, 2 left lung cases and 10 right lung cases. Of the 6 patients who underwent surgical resection, five cases were confined to lung tissue (T1), one case had mediastinal invasion (T3), two cases had regional lymph node metastasis (N1), and none had distal metastasis. Microscopically, 11 cases showed monophasic spindle cell type and one case showed biphasic type composed of mainly epithelial cells consisting of cuboidal to columnar cells with glandular and cribriform structures. It was difficult to make the diagnosis by using the biopsy specimens. Immunohistochemistry (IHC) showed CKpan expression in 8 of 12 cases; EMA expression in 11 of 12 case; TLE1 expression in 8 of 12 cases; S-100 protein expression in two of 12 cases; various expression of bcl-2 and vimentin in 12 cases, but no expression of SOX10 and CD34 in all the cases. The Ki-67 index was 15%-30%. The expression of SS18-SSX fusion antibody was diffusely and strongly positive in all 12 cases. SOX2 was partially or diffusely expressed in 8 of 12 cases, with strong expression in the epithelial component. H3K27Me3 was absent in 3 of 12 cases. SS18 gene translocation was confirmed by fluorescence in situ hybridization (FISH) test in all 12 samples. Six cases underwent surgery and postoperative chemotherapy, while the other six cases had chemotherapy alone. Ten patients were followed up after 9-114 months, with an average of 41 months and a median of 26 months. Five patients survived and five died of the disease within two years. Conclusions: PSSL is rare and has a broad morphological spectrum. IHC and molecular tests are needed for definitive diagnosis. Compared with current commonly used IHC markers, SS18-SSX fusion antibody has better sensitivity to PSSL, which could be used as an alternative for FISH, reverse transcription-polymerase chain reaction or next generation sequencing in the diagnosis of PSSL.
Male ; Female ; Humans ; Adult ; Middle Aged ; Aged ; Biomarkers, Tumor/analysis* ; Sarcoma, Synovial/diagnosis* ; In Situ Hybridization, Fluorescence ; Histones/genetics* ; Proto-Oncogene Proteins/metabolism* ; Oncogene Proteins, Fusion/genetics* ; Repressor Proteins/metabolism* ; Lung/pathology* ; Lung Neoplasms

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

A calcification mass was incidentally found in the soft tissue of a patient who had a history of trauma to the extremity during examination. The patient had no symptom. The pathological analysis of the mass revealed it was an early-phase synovial sarcoma (SS). The diagnosis was made before the onset of symptoms and proper surgical intervention was performed. Therefore, in case of a <1 cm lesion clinically suspicious of myositis ossificans, SS should be taken into consideration as a possible diagnosis.
Diagnosis ; Extremities ; Femur ; Humans ; Myositis Ossificans ; Myositis ; Sarcoma, Synovial

Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma.
Bone Neoplasms/secondary ; Carcinoma, Renal Cell/pathology/radiography ; Diagnosis, Differential ; Fibrosarcoma/radiography ; Histiocytoma/radiography ; Humans ; Kidney Neoplasms/*pathology/radiography ; Leiomyosarcoma/pathology/radiography ; Magnetic Resonance Imaging ; Middle Aged ; Neuroectodermal Tumors, Primitive/pathology/radiography ; Osteosarcoma/pathology ; Sarcoma ; Sarcoma, Synovial/radiography ; Tomography, X-Ray Computed

BACKGROUND: We aimed to describe the clinical characteristics and outcomes of unplanned excisions of synovial sarcomas. METHODS: In total, 90 patients with synovial sarcomas in the extremities were retrospectively reviewed. Patients were divided into unplanned excision (n = 38) and planned excision (n = 52) groups. The average follow-up period was 6 years. The clinicopathological characteristics and oncologic outcomes were compared. RESULTS: The unplanned excision group showed longer duration of symptoms before diagnosis (p = 0.023), smaller lesion dimensions (p = 0.001), superficial location (p = 0.049), and predilection in the upper extremities (p = 0.037). Synovial sarcomas were most commonly misdiagnosed as neurogenic tumors (56%) in the upper extremities or as cystic masses (47%) in the lower extremities. Oncological outcomes, including disease-specific survival, metastasis-free survival, or local recurrence were not significantly different between the 2 groups (p = 0.159, p = 0.444, and p = 0.335, respectively). Repeated unplanned excision (p = 0.012) and delayed re-excision (p = 0.038) were significant risk factors for local recurrence in the unplanned excision group. CONCLUSIONS: Synovial sarcomas treated with unplanned excision had distinct characteristics. These findings are important for developing diagnostic and therapeutic strategies for synovial sarcoma.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Sarcoma, Synovial/*diagnosis/*surgery ; Soft Tissue Neoplasms/*diagnosis/*surgery ; Treatment Outcome ; Young Adult

Small round cell tumors (SRCTs) are a heterogeneous group of neoplasms composed of small, primitive, and undifferentiated cells sharing similar histology under light microscopy. SRCTs include Ewing sarcoma/peripheral neuroectodermal tumor family tumors, neuroblastoma, desmoplastic SRCT, rhabdomyosarcoma, poorly differentiated round cell synovial sarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, small cell malignant peripheral nerve sheath tumor, and small cell schwannoma. Non-Hodgkin\'s malignant lymphoma, myeloid sarcoma, malignant melanoma, and gastrointestinal stromal tumor may also present as SRCT. The current shift towards immunohistochemistry and cytogenetic molecular techniques for SRCT may be inappropriate because of antigenic overlapping or inconclusive molecular results due to the lack of differentiation of primitive cells and unavailable genetic service or limited moleculocytogenetic experience. Although usage has declined, electron microscopy (EM) remains very useful and shows salient features for the diagnosis of SRCTs. Although EM is not always required, it provides reliability and validity in the diagnosis of SRCT. Here, the ultrastructural characteristics of SRCTs are reviewed and we suggest that EM would be utilized as one of the reliable modalities for the diagnosis of undifferentiated and poorly differentiated SRCTs.
Chondrosarcoma, Mesenchymal ; Cytogenetics ; Diagnosis ; Gastrointestinal Stromal Tumors ; Genetic Services ; Humans ; Immunohistochemistry ; Lymphoma ; Melanoma ; Microscopy ; Microscopy, Electron ; Microscopy, Electron, Transmission* ; Neurilemmoma ; Neuroblastoma ; Neuroectodermal Tumors ; Osteosarcoma ; Pathology ; Peripheral Nerves ; Reproducibility of Results ; Rhabdomyosarcoma ; Sarcoma, Myeloid ; Sarcoma, Synovial

Soft tissue tumors are classified into benign and malignant on the basis of the patient's age, medical history, physical examination, pathological and radiologic examination. We have to caution against misdiagnosis of malignant tumor which can delay the treatment time. Lipoma, schwannoma, hemangioma, and ganglion cysts are common benign tumors, usually of small size and are often located in the superficial layer. Although it may be suspected as a benign tumor, performing contrast-enhanced magnetic resonance maging is preferably advantageous. Liposarcoma and undifferentiated pleomorphic sarcoma, the most common malignant soft tissue tumors, usually occur after middle age; rhabdomyosarcoma is usually presented in children and synovial sarcoma often occurs at a younger age. The magnetic resonance (MR) signal intensity of lipoma shows uniformity with subcutaneous fat, sarcoma should be suspected if it has a contrast-enhanced and non-fat-suppressed part. The MR signals of ganglion cysts show homogeneous and same signal intensity with joint fluid and urine, while the liquid containing sarcoma, like synovial sarcoma, is characterized by heterogeneous signal intensity and contrast enhancement. If surgery is performed, an incision should be made in the longitudinal direction of the limb and the excised tumor should be sent for pathology analysis. When the macroscopic finding of the tumor during surgery is different from the expected diagnosis, the operation should cease with biopsy only or the small superficial tumor can be excised widely if possible. The transfer should be considered unless you can be sure of a benign tumor in hands and feet of children. When diagnosed as malignant tumors, patients should be provided with sufficient information that can lead them to a musculoskeletal tumor specialist.
Biopsy ; Child ; Diagnosis* ; Diagnostic Errors ; Extremities ; Foot ; Ganglion Cysts ; Hand ; Hemangioma ; Humans ; Joints ; Lipoma ; Liposarcoma ; Middle Aged ; Neurilemmoma ; Pathology ; Physical Examination ; Rhabdomyosarcoma ; Sarcoma ; Sarcoma, Synovial ; Specialization ; Subcutaneous Fat

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

Synovial sarcoma (SS) is a highly malignant tumor that accounts for 10% of all soft-tissue sarcomas. Primary SS arising from the lung is extremely rare, and the prognosis is poor. We report a case of pulmonary SS presenting with a mass lesion invading the right upper and middle lobes, extending to the mediastinum and the chest wall. After tru-cut biopsy, surgical resection was performed. The final diagnosis was SS (biphasic type) based on histological and immunohistochemical findings. There are no guidelines for optimal treatment due to the rarity of these tumors. Current treatment includes surgery and adjuvant chemotherapy and/or radiotherapy.
Biopsy ; Chemotherapy, Adjuvant ; Diagnosis ; Immunohistochemistry ; Lung* ; Mediastinum ; Prognosis ; Radiotherapy ; Sarcoma ; Sarcoma, Synovial* ; Thoracic Wall

We report a case of intramuscular hemangioma in the subscapularis muscle and the resulting impairment of shoulder function in an adult patient. A nineteen-year-old female complained of shoulder pain and the development of a mass in the absence of previous trauma. Physical examinations, including lift-off and belly-press tests, showed abnormality. X-ray showed multiple calcifications in the front of the scapula. Magnetic resonance imaging showed a soft-tissue mass occupying almost the entire intramuscular portion of the subscapularis muscle. An arthroscopic examination excluded the possibility of a joint invasion, after which the entire mass was successfully removed by open excision. The displacement of the subscapularis by the mass was relieved after the surgery. Pathological diagnosis of the tissue confirmed a cavernous hemangioma. Both shoulder pain and function was improved after operation. There was no evidence of recurrence even at the 2-year follow-up. Rare forms of hemangioma adjacent to the shoulder joint could be successfully managed with surgical excision. Differential diagnosis, such as synovial chondromatosis, pigmented villonodular synovitis, and malignant sarcoma, should also be considered.
Adult ; Chondromatosis, Synovial ; Diagnosis ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioma ; Hemangioma, Cavernous* ; Humans ; Joints ; Magnetic Resonance Imaging ; Physical Examination ; Recurrence ; Rotator Cuff ; Sarcoma ; Scapula ; Shoulder ; Shoulder Joint ; Shoulder Pain ; Synovitis, Pigmented Villonodular