Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.
Adult ; Female ; Humans ; Male ; Middle Aged ; Chondrosarcoma/surgery* ; Fibrosarcoma ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skull Base/surgery* ; Skull Base Neoplasms/surgery* ; Temporal Bone/pathology* ; Treatment Outcome

Humans ; Fibrosarcoma/surgery* ; Sarcoma ; Temporal Bone ; Soft Tissue Neoplasms/surgery*

Humans ; Solitary Fibrous Tumors/pathology* ; Osteosarcoma/pathology* ; Fibrosarcoma ; Bone Neoplasms/surgery*

Fibrosarcoma/surgery* ; Humans ; Infant, Newborn ; Lung Neoplasms ; Neck

Actins ; metabolism ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Calcium-Binding Proteins ; metabolism ; Carcinoma ; metabolism ; pathology ; Cyclophosphamide ; therapeutic use ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Fibrosarcoma ; metabolism ; pathology ; Fluorouracil ; therapeutic use ; Follow-Up Studies ; Humans ; Leiomyosarcoma ; drug therapy ; metabolism ; pathology ; surgery ; Mastectomy, Segmental ; Methotrexate ; therapeutic use ; Microfilament Proteins ; metabolism ; Neurilemmoma ; metabolism ; pathology ; Phyllodes Tumor ; metabolism ; pathology ; Postoperative Period ; Vimentin ; metabolism

12E7 Antigen ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Diagnosis, Differential ; Fibrosarcoma ; metabolism ; pathology ; Heart Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Humans ; Male ; Mesothelioma ; genetics ; metabolism ; pathology ; Middle Aged ; Mucin-1 ; metabolism ; Oncogene Proteins, Fusion ; metabolism ; Pericardiectomy ; Pericardium ; pathology ; Sarcoma ; metabolism ; pathology ; Sarcoma, Synovial ; genetics ; metabolism ; pathology ; surgery ; Translocation, Genetic ; Vimentin ; metabolism

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Female ; Fibroma ; pathology ; Fibrosarcoma ; pathology ; Follow-Up Studies ; Humans ; Hysterectomy ; Inflammation ; Keratins ; metabolism ; Leiomyoma ; pathology ; Neoplasm Recurrence, Local ; Neoplasms, Muscle Tissue ; drug therapy ; metabolism ; pathology ; surgery ; Receptor Protein-Tyrosine Kinases ; metabolism ; Uterine Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult

Actins ; metabolism ; Adult ; Breast Diseases ; metabolism ; pathology ; surgery ; Breast Neoplasms ; pathology ; Calcium-Binding Proteins ; metabolism ; Carcinoma ; pathology ; Diagnosis, Differential ; Fasciitis ; metabolism ; pathology ; surgery ; Female ; Fibroma ; pathology ; Fibrosarcoma ; pathology ; Follow-Up Studies ; Humans ; Microfilament Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the disease spectrum, clinical and pathologic features of primary cardiac neoplasms at a single medical institution during a period of eight years.

METHODSThe clinical and pathologic features of 81 cases of primary cardiac neoplasms encountered at the Affiliated Hospital of Medical College, Qingdao University from January, 2004 to December, 2011, either for cardiovascular surgery or as pathology consultation cases, were retrospectively reviewed. Immunohistochemical study was carried out in selected examples.

RESULTSAmongst the 81 cases studied, 73 cases (90.1%) were benign and 8 cases (9.9%) were malignant. Cardiac myxomas accounted for 97.3% (71/73) of all the benign cases. Six of the 8 malignant cases represented soft tissue sarcomas. The commonest presenting symptom was dyspnea. The histologic subtypes included myxoma (number = 71), angiosarcoma (number = 2), malignant fibrous histiocytoma (number = 2), hemangioma (number = 1), liposarcoma (number = 1), lymphoma (number = 1), myxofibrosarcoma (number = 1) and papillary fibroelastoma (number = 1).

CONCLUSIONSWhile the majority of primary cardiac neoplasms are myxomas, other tumor types do exist. Accurate histologic diagnosis and timely surgery are crucial in patient management.

Adolescent ; Adult ; Aged ; Child ; Female ; Fibroma ; metabolism ; pathology ; surgery ; Fibrosarcoma ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Heart Neoplasms ; metabolism ; pathology ; surgery ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; metabolism ; pathology ; surgery ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Liposarcoma ; metabolism ; pathology ; surgery ; Lymphoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Myxoma ; metabolism ; pathology ; surgery ; Sarcoma ; metabolism ; pathology ; surgery ; Treatment Outcome ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicopathologic features, immunophenotypes and differential diagnosis of myxoinflammatory fibroblastic sarcoma (MIFS).

METHODSThe clinical and pathologic features of 6 cases of MIFS were analyzed. Immunohistochemical study was performed using the standard EnVision method.

RESULTSThere were altogether 2 adult males and 4 adult females (median age = 47 years and mean age = 50 years). Three cases were located in the lower extremities, 2 in the upper limbs and 1 in the axillary region. Common presentation included slowly growing mass or swelling in the extremities, accompanied by mild pain or tenderness. Grossly, the tumor appeared multinodular and ranged from 2.5 cm to 4.6 cm in diameter (mean = 3.4 cm). Microscopically, there was a dense inflammatory infiltrate merging with hyaline and myxoid zones in various proportions. Spindle-shaped tumor cells were seen admixed with large atypical cells which distributed singly or in small clusters, amongst an inflammatory, hyaline or a myxoid background. These atypical cells had large nuclei and prominent nucleoli, resembling virocytes, Reed-Sternberg cells or ganglion cells. Mitotic figures were rarely identified. Extracellular mucin associated with scattered monovacuolated or multivacuolated lipoblast-like cells was noted. Immunohistochemically, these bizarre cells were consistently positive for vimentin, but negative for a panel of antibodies including LCA, CD15, CD30, CD34, CD68, S-100, HMB45, AE1/AE3, smooth muscle actin and desmin. Follow-up result was available in 4 cases; and 2 of them showed local recurrence after an incomplete excision. There was no evidence of distant metastasis.

CONCLUSIONSMISF is a low-grade sarcoma of fibroblastic differentiation. Awareness of the clinical and pathologic characteristics is helpful in arriving at the correct diagnosis and distinction from benign inflammatory fibromyxoid lesions.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Extremities ; Female ; Fibroblasts ; pathology ; Fibrosarcoma ; metabolism ; pathology ; surgery ; Follow-Up Studies ; Humans ; Inflammation ; pathology ; Male ; Middle Aged ; Myxosarcoma ; metabolism ; pathology ; surgery ; Neoplasm Recurrence, Local ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism