Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.
Adolescent ; Adult ; Biomarkers, Tumor ; Child ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Hemangioendothelioma, Epithelioid/pathology* ; Hemangioma ; Histiocytoma, Malignant Fibrous/diagnosis* ; Humans ; Male ; Pain ; Precancerous Conditions/diagnosis*

Humans ; RNA-Binding Protein EWS/genetics* ; Histiocytoma, Malignant Fibrous/pathology* ; Oncogene Proteins, Fusion/genetics* ; Biomarkers, Tumor ; Cyclic AMP Response Element-Binding Protein/genetics*

OBJECTIVE: To explore the efficacy of the resection of periacetabular malignant tumors and the reconstruction with modular endoprosthesis.
 METHODS: From August 2006 to December 2012, 22 patients with periacetabular malignant tumors, who received the resection and reconstruction with modular prosthesis, were retrospectively reviewed. There were 11 males and 11 females, and the average age was 44 (16-65) years old. Pathological results showed there were 13 cases of chondrosarcoma, 5 cases of osteosarcoma, 2 cases of Ewing's sarcoma, 1 case of maligant fibrous histiocytoma, and 1 case of giant cell tumor. According to the classification system by Enneking, there were 11 cases of Type II+III resection, 5 cases of Type I+II+III resection, 3 cases of Type I+II resection, and 3 cases of Type II resection.
 RESULTS: All patients were followed up. The average time for follow-up was 49 (11-103) months. At the last time of follow-up, 13 patients (59%) were still alive, 9 patients (41%) died due to their primary disease. Metastasis occurred in 8 patients (36%), and local recurrence occurred in 5 patients (23%). The mean Musculoskeletal Tumor Society (MSTS) score for 13 cases of alive patients at the latest time of follow-up was (18.5±5.7) months. The mean score for 11 patients, whose limb salvage were successful, was 20.7±2.0.
 CONCLUSION Reconstruction with modular prosthesis after wide resection for periacetabular malignant tumor can achieve satisfied outcome.
Acetabulum ; pathology ; surgery ; Adolescent ; Adult ; Aged ; Bone Neoplasms ; mortality ; surgery ; Chondrosarcoma ; mortality ; surgery ; Female ; Giant Cell Tumors ; mortality ; surgery ; Hip Prosthesis ; Histiocytoma, Malignant Fibrous ; mortality ; surgery ; Humans ; Limb Salvage ; Male ; Middle Aged ; Neoplasm Metastasis ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Osteosarcoma ; mortality ; surgery ; Prosthesis Implantation ; statistics & numerical data ; Retrospective Studies ; Sarcoma, Ewing ; mortality ; surgery ; Treatment Outcome

OBJECTIVETo study the clinicopathologic characteristics and diagnostic criteria of interdigitating dendritic cell sarcoma/tumor (IDCS/T).

METHODSThe clinical features, histologic findings and results of immunohistochemical study in six cases of IDCS/T were analyzed, with review of literature.

RESULTSThe age of patients ranged from 20 to 68 years. The sites of involvement included lymph node, tonsil and soft tissue. Histologically, the tumor cells were arranged in sheets, fascicles or whorls and intimately admixed with abundant lymphocytes and plasma cells. They were oval to spindly in shape and contained pale eosinophilic cytoplasm, oval nuclei and distinct nucleoli.Immunohistochemical study showed that the tumor cells were positive for S-100 protein and CD68.

CONCLUSIONSIDCS/T is a rare malignant tumor with poor prognosis. It carries distinctive histologic pattern and immunophenotype. The entity needs to be distinguished from follicular dendritic cell sarcoma/tumor, anaplastic large cell lymphoma and other spindle cell sarcomas in occurring soft tissue.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Dendritic Cell Sarcoma, Interdigitating ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Humans ; Lymph Nodes ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Male ; Middle Aged ; Neck ; S100 Proteins ; metabolism ; Sarcoma ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; Thigh ; Tonsillar Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

Ifosfamide-induced Fanconi syndrome is a rare complication that typically occurs in young patients due to a cumulative dose of ifosfamide > 40-60 g/m2, a reduction in kidney mass, or concurrent cisplatin treatment. It is usually characterized by severe and fatal progression accompanied by type II proximal renal tubular dysfunction, as evidenced by glycosuria, proteinuria, electrolyte loss, and metabolic acidosis. Diabetes insipidus is also a rare complication of ifosfamide-induced renal disease. We herein describe a case involving a 61-year-old man who developed ifosfamide-induced Fanconi syndrome accompanied by diabetes insipidus only a few days after the first round of chemotherapy. He had no known risk factors. In addition, we briefly review the mechanisms and possible therapeutic options for this condition based on other cases in the literature. Patients who receive ifosfamide must be closely monitored for renal impairment to avoid this rare but fatal complication.
Acidosis/chemically induced ; Antineoplastic Agents, Alkylating/*adverse effects ; Chemotherapy, Adjuvant ; Diabetes Insipidus/*chemically induced/diagnosis/therapy ; Fanconi Syndrome/*chemically induced/diagnosis/therapy ; Fatal Outcome ; Histiocytoma, Malignant Fibrous/*drug therapy/pathology ; Humans ; Ifosfamide/*adverse effects ; Male ; Middle Aged ; Neoadjuvant Therapy/*adverse effects ; Time Factors

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Heart Atria ; pathology ; Histiocytoma, Malignant Fibrous ; diagnosis ; Humans ; Male ; Middle Aged ; Young Adult

OBJECTIVETo investigate the clinicopathologic characteristics, differential diagnosis and biologic behaviors of pleomorphic rhabdomyosarcoma (PRMS).

METHODSThe clinical findings, pathological features and immunophenotypes were reviewed in 44 cases of PRMS (encountered during the period from 2005 to 2012). The clinical outcome was analyzed.

RESULTSThere were 33 males and 11 females with age ranging from 2 to 85 years (mean, 51 years; median, 55 years). Of 44 tumors, 22 occurred in the extremities (50.0%), 16 in the trunk (36.4%), 5 in the internal organs (11.4%), and 1 in the head and neck (2.2%). Histologically, 40 tumors showed features of pleomorphic sarcoma with striking resemblance to undifferentiated pleomorphic sarcoma (UPS)/malignant fibrous histiocytoma(MFH). However, variable amount of pleomorphic rhabdomyoblasts (PRMB) were identified in most cases. The remaining 4 tumors were composed predominantly of fascicles of spindle cells with interspersed PRMBs. Immunohistiochemically, tumor cells showed diffuse staining of desmin (41/41,100%), with variable expression of myogenin (18/32, 56.3%), MyoD1 (10/21, 47.6%) and MSA (21/29, 72.4%), whereas α-SMA was negative in most cases. Follow-up data (range, 2 to 51 months) available in 29 cases showed 12 patients were alive with unresectable or recurrent disease and 17 patients were alive with no evidence of disease. The median disease-free and overall survivals was 6.0 months (mean, 9.1 months) and 8.0 months (mean, 11.2 months) respectively. Thirteen patients (44.8%) exhibited progression of disease with recurrence in 4 cases and metastasis in 9 cases. The median interval to progression was 6.0 months (mean, 5.9 months).

CONCLUSIONSThe presence of pleomorphic cells with strong eosinphilic cytoplasm in a pleomorphic sarcoma is suggestive of a PRMS. Diffuse, strong expression of desmin and negative staining for α-SMA further facilitate the diagnosis of PRMS and its differential diagnosis from pleomorphic leiomyosarcoma. Although PRMS may affect children or adolescents, it should be cautious not to misdiagnose anaplastic rhabdomyosarcoma as PRMS. PRMS is a high-grade sarcoma with a poor prognosis.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Chemoradiotherapy, Adjuvant ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Desmin ; metabolism ; Diagnosis, Differential ; Extremities ; Female ; Follow-Up Studies ; Histiocytoma, Malignant Fibrous ; metabolism ; pathology ; Humans ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Myogenin ; metabolism ; Neoplasm Recurrence, Local ; Retrospective Studies ; Rhabdomyosarcoma ; metabolism ; pathology ; secondary ; surgery ; therapy ; Survival Rate ; Young Adult

Actins ; metabolism ; Dermatofibrosarcoma ; classification ; pathology ; Desmin ; metabolism ; Head and Neck Neoplasms ; metabolism ; pathology ; Histiocytoma, Benign Fibrous ; classification ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; classification ; metabolism ; pathology ; Humans ; Oncogene Proteins, Fusion ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; Vimentin ; metabolism ; Xanthomatosis ; pathology

OBJECTIVETo investigate the clinical pathological features of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP).

METHODSThe clinical history, histopathological features and immunohistochemical characteristics were analyzed in twelve cases of FS-DFSP from January 1997 to February 2011, and related literature were reviewed.

RESULTSAge of the patients (2 females, 10 males) at diagnosis ranged from 41 to 70 years (mean 53 years). Among the 12 cases of FS-DFSP, 9 cases aroused in recurrent ordinary DFSP. Histologically, FS areas in FS-DFSP were characterized by a fascicular and highly cellular histology, frequently showing a characteristic herringbone pattern. FS-DFSP showed diminishment of CD34 staining in FS areas. The labeling index of Ki-67 was much higher in the FS areas (10%-40%) than that in the conventional DFSP areas (2%-5%). All the patients were treated by operation with local excision or wide excision. Postoperative radiotherapy and chemotherapy was administered in two cases respectively. Follow-up information in 9 of 12 patients (9 to 86 months) revealed local recurrence in 6 patients. Distant metastases were seen in two patients. One patient was died in the follow up period.

CONCLUSIONSFS-DFSP is a rare and unique subtype of DFSP and is associated with significant elevated risk of both local and distance metastasis, usually followed by poor outcome. Compared to ordinary DFSP as a borderline neoplasm, FS-DFSP should be considered as a malignant tumor.

Adult ; Aged ; Antigens, CD34 ; metabolism ; Chemotherapy, Adjuvant ; Dermatofibrosarcoma ; metabolism ; pathology ; therapy ; Diagnosis, Differential ; Female ; Fibroma ; pathology ; Fibrosarcoma ; metabolism ; pathology ; therapy ; Follow-Up Studies ; Histiocytoma, Benign Fibrous ; metabolism ; pathology ; Histiocytoma, Malignant Fibrous ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; secondary ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Radiotherapy, Adjuvant ; Retrospective Studies ; Skin Neoplasms ; metabolism ; pathology ; therapy

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