OBJECTIVETo study the clinicopathologic characteristics of perivascular epithelioid cell tumor (PEComa), not otherwise specified (NOS) and to evaluate the diagnostic criteria for malignancy.

METHODSThe clinical and pathologic features of 31 cases of PEComa-NOS were reviewed. The follow-up data available were analyzed.

RESULTSThere were a total of 24 females and 7 males. The age of the patients ranged from 13 to 66 years (mean = 40 years). The site of tumor occurrence included gynecologic organs (n = 12), intraabdominal/peritoneal soft tissue (n = 10), gastrointestinal tract (n = 4), thigh (n = 2), mediastinum (n = 1), left groin (n = 1) and urinary bladder (n = 1). None of the cases was associated with tuberous sclerosis complex. Histologic examination showed that 23 cases (74%) were clear cell sugar tumor-like, 4 cases (13%) were clear cell myomelanocytic tumor-like and 4 cases (13%) were of mixed epithelioid-spindled morphology. According to the classification system proposed by Folpe et al, 19 cases (61%) were classified as malignant, 7 cases (23%) as PEComa of uncertain malignant potential and 5 cases (16%) as benign. The expression rates of HMB45, smooth muscle actin and desmin in tested cases were 100% (31/31), 67% (14/21) and 6/18, respectively. Follow-up data (1 to 56 months) were available in 23 cases (74%). Amongst the 16 cases of malignant PEComa, 7 patients were still alive with no evidence of disease, 6 patients were alive with unresectable or recurrent/metastatic disease and 3 patients died of the disease. The local recurrence and metastasis in those 16 cases were 6 cases and 5 cases, respectively. One of the 4 patients with PEComa of uncertain malignant potential died, while the remaining 3 patients and all of the patients with benign PEComa had an uneventful clinical course.

CONCLUSIONSThe classification system of PEComas proposed by Folpe et al. is reliable in routine practice. Correlation with the clinical and radiologic findings however is prudent when dealing with core biopsy specimens or sampling from exploration laparotomy. Owing to the histologic heterogeneity of this entity, thorough understanding of the morphologic spectrum is essential in arriving at a correct diagnosis.

Abdominal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Adolescent ; Adult ; Aged ; Desmin ; metabolism ; Female ; Follow-Up Studies ; Gastrointestinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Genital Neoplasms, Female ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Lymphatic Metastasis ; Male ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Perivascular Epithelioid Cell Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Prognosis ; Young Adult

The distinction of a spitz nevus from a melanoma can be difficult and in some cases, impossible. A misdiagnosed spitz nevus can metastasize and lead to fatal outcomes, especially in children. A 5-yr-old girl presented with a 1-yr history of a solitary pinkish nodule on her left hand. On physical examination, she had a palpable left axillary lymph node. We performed biopsy and checked 3 sentinel lymph nodes (SLN) on her axillary area. The biopsy specimen showed multiple variably sized and shaped nests with large spindle or polygonal cells and SLN biopsy showed 3 of 3 lymph nodes that were metastasized. Under the diagnosis of spitzoid melanoma, she was treated with excision biopsy and complete left axillary lymph nodes were dissected. She received interferon-alpha2b subcutaneously at a dose of 8 MIU per day, 3 times weekly for 12 months, and shows no recurrence.
Antineoplastic Agents/therapeutic use ; Child, Preschool ; Female ; Humans ; Interferon-alpha/therapeutic use ; Lymphatic Metastasis ; Melanoma/drug therapy/*pathology/surgery ; *Nevus, Epithelioid and Spindle Cell ; Recombinant Proteins/therapeutic use ; Skin Neoplasms/drug therapy/*pathology/surgery

OBJECTIVE: To investigate the clinopathological characteristics, differential diagnosis, therapy and prognosis of sinonasal mucosal malignant melanoma. METHOD: Clinopathological data of 18 cases which were diagnosed by pathology and immmunohistochemistry were analyzed retrospectively. All cases were proved by pathology and immmunohistochemistry. All cases were performed operations. 5 underwent single surgery. 4 underwent surgery plus adjuvant radiotherapy. 4 underwent surgery plus adjuvant radiotherapy chemotherapy. 5 underwent surgery plus adjuvant chemoradiation. RESULT: All cases were followed up for a period of 1 to 7 years after operation. Twelve patients died of tumor until the last follow-up, meanwhile 6 patients stayed alive. In Six cases recurrence occurred. In five casescervical lymph node metastasis occurred, of which 3 cases received neck dissection and 2 cases received chemotherapy and radiotherapy due to no surgical indications. In three cases distant metastasis oc- curred. CONCLUSION Sinonasal mucosal malignant melanoma is rare and highly heterogenous. Current diagnosis depends on clinical characteristics and immunohistochemical examination. It still should be differentially diagnosed from other tumors. CT and MRI image examination can provide some helpful information to understand the extent and nature of lesions. The treatment of nasal endoscopic or the surgery under endoscopy has become to be a safe, viable and reasonable alternative to open resection. Appropriate indication must be carefully selected for these lesions.
Chemotherapy, Adjuvant ; Endoscopy ; Humans ; Lymphatic Metastasis ; Melanoma ; drug therapy ; pathology ; surgery ; Mucous Membrane ; Neck Dissection ; Neoplasm Recurrence, Local ; Nose Neoplasms ; Paranasal Sinus Neoplasms ; drug therapy ; pathology ; surgery ; Prognosis ; Radiotherapy, Adjuvant ; Retrospective Studies

Adnexa Uteri ; surgery ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Melanoma ; drug therapy ; pathology ; secondary ; surgery ; Melanosis ; pathology ; Middle Aged ; Ovarian Neoplasms ; drug therapy ; pathology ; surgery ; Pelvic Neoplasms ; secondary ; Peritoneal Neoplasms ; secondary ; Teratoma ; drug therapy ; pathology ; secondary ; surgery

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma ; metabolism ; pathology ; Cyclophosphamide ; therapeutic use ; Dendritic Cell Sarcoma, Follicular ; drug therapy ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Head and Neck Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lung Neoplasms ; drug therapy ; secondary ; Male ; Melanoma ; metabolism ; pathology ; Middle Aged ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Vincristine ; therapeutic use

OBJECTIVETo investigate the treatment and prognosis of the patients with oral mucosal melanoma (OMM).

METHODSThe clinicopathological and follow-up data of patients with OMM in Sun Yat-sen University Cancer Center from January 1976 to December 2005 were analyzed retrospectively.

RESULTSFifty-one cases were analyzed. The pathological lymph node metastasis rate was 61% (31/51) and the affected sites were confined to level I(b)-III (94%). The overall three year and five yearsurvival rates were 35% and 21% respectively. No significant difference of three year and five year survival rates were found between the group of incisional biopsy and the group of excisional biopsy. The prognosis was not affected by pigmentation. The survival rate of the patients receiving surgery combined with biotherapy or biochemotherapy was significantly higher than that of the patients treated by other modalities (P = 0.003).

CONCLUSIONSIn patients with OMM, lymph node metastasis was mostly confined to level I(b)-III. Incisional biopsy and pigmentation were not associated with an unfavorable prognosis. The prognosis of the patients with OMM was poor and the patients may get a better prognosis by receiving surgery combined with biotherapy or biochemotherapy.

Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; BCG Vaccine ; therapeutic use ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Interferon-gamma ; therapeutic use ; Interleukin-2 ; therapeutic use ; Lung Neoplasms ; secondary ; Lymph Node Excision ; Lymphatic Metastasis ; Male ; Melanoma ; drug therapy ; pathology ; surgery ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Mouth Mucosa ; pathology ; surgery ; Mouth Neoplasms ; drug therapy ; pathology ; surgery ; Retrospective Studies ; S100 Proteins ; metabolism ; Survival Rate

OBJECTIVETo study the morphologic features, immunophenotypes, differential diagnoses and prognosis of histiocytic sarcoma (HS).

METHODSThe clinical and pathologic findings of 6 cases of HS were reviewed. Immunohistochemical assay (Elivision staining) was also performed. Follow-up information was available in 4 patients.

RESULTSThere were altogether 3 males and 3 females. The age of patients ranged from 12 to 81 years old (median = 54.6 years). The sites of involvement included lymph node (number = 2 cases) and skin or soft tissue (number = 4 cases). The tumor was composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin and large nucleoli. Binucleated form was not uncommon. Two of the cases showed increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 3 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. Mitotic figures were readily identified. The tumor cells were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for leukocyte common antigen, CD4, CD68 and CD163. Four of the 5 cases studied also expressed lysozyme. Amongst the 4 patients with follow-up information available, 3 died of the disease at 6 to 11 months interval after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived for 3 years, with no evidence of tumor recurrence.

CONCLUSIONSAccurate diagnosis of the HS is based on the combination of morphologic examination and immunohistochemical assay. HS often presents with clinically advanced disease and pursues an aggressive clinical course, with a poor response to therapy. However, a subset of cases presenting with clinically localized lesion may carry a relatively favorable long-term outcome.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Child ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Melanoma ; metabolism ; pathology ; Muramidase ; metabolism ; Prognosis ; Receptors, Cell Surface ; metabolism ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult

No abstract available.
Antigens, Neoplasm/metabolism ; Bone Neoplasms/diagnosis/drug therapy/secondary ; Disease Progression ; Humans ; Interferons/therapeutic use ; Liver Neoplasms/diagnosis/drug therapy/secondary ; Male ; Melanoma/*diagnosis/pathology/surgery ; Middle Aged ; Neoplasm Proteins/metabolism ; Positron-Emission Tomography ; Rectal Neoplasms/*diagnosis/pathology/surgery ; S100 Proteins/metabolism ; Tamoxifen/therapeutic use ; Tomography, X-Ray Computed