Pediatric solid tumors have many similarity among different tumors. These tumors present small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the investigation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumor. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is an useful tool for a rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.
Cell Line ; Diagnosis* ; Diagnosis, Differential ; Humans ; Lymphoma ; Pathology ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar ; RNA ; Sarcoma ; Sarcoma, Ewing ; Translocation, Genetic ; Wilms Tumor

Abscess ; pathology ; Acute Disease ; Female ; Humans ; Infant ; Mastoid ; pathology ; Otitis Media ; diagnosis ; Rhabdomyosarcoma ; diagnosis

Report a case of cervical rhabdomyosarcoma. The illness development is rapid and the prognosis is poor. The clinical manifestations of dysfunction caused by local mass increases rapidly. The relevant imaging examination and laboratory examination can help establish a preliminary diagnosis, pathological examination of tumor can make a clear diagnosis.
Head and Neck Neoplasms ; diagnosis ; pathology ; Humans ; Male ; Neck ; Prognosis ; Rhabdomyosarcoma ; diagnosis ; pathology

Rhabdomyosarcoma is a kind of skeletal muscle ifferentiation tendency of primitive mesenchymal malignant tumor. It is common in pediatric malignant pleomorphic rhabdomyosarcoma, and its prognosis is poor. It is easy to relapse, and its predilection sites in head and face were around the eyes, the external auditory meatus, nasal cavity and paranasal sinuses. We should take comprehensive treatment including surgical operation, radiotherapy and chemotherapy drug combination for embryonal rhabdomyosarcoma.
Child ; Ear Canal ; pathology ; Humans ; Infant ; Nasal Cavity ; pathology ; Orbit ; pathology ; Paranasal Sinuses ; pathology ; Prognosis ; Rhabdomyosarcoma, Embryonal ; diagnosis ; therapy

OBJECTIVE: To study the clinicopathological characteristics, diagnosis and differential diagnosis of hypopharyngeal carcinosarcoma. METHOD: Clinical data of one case with hypopharyngeal carcinosarcoma proved by pathology were analyzed retrospectively. The related literatures were reviewed. RESULT: The masses were surgically removed,and the postoperative diagnosis was confirmed to be carcinosarcoma by histopathological examination. After radiotherapy and chemotherapy, the patient recovered well postoperatively without recurrence during 18-month follow-up. CONCLUSION Carcinosarcoma of the hypopharynx is an extremely rare malignant tumor with distinctive histological, clinical and immunohistochemical features. The final diagnosis depends on histopathology. This tumor should be differentiated from other tumors such as sarcomatoid carcinoma and the reaction of radiotherapy of carcinoma. Surgery is the proper treatment strategy for carcinosarcoma of the hypopharynx. The patients with locally advanced, postoperative residual tumor or tumor without clear safe surgical margin should undergo radiotherapy, and the postoperative follow up should be in time.
Carcinoma, Squamous Cell ; diagnosis ; pathology ; Carcinosarcoma ; diagnosis ; pathology ; Humans ; Hypopharyngeal Neoplasms ; diagnosis ; pathology ; Hypopharynx ; pathology ; Male ; Middle Aged ; Rhabdomyosarcoma ; diagnosis ; pathology

A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
Biopsy, Needle ; Burkitt Lymphoma/*diagnosis/pathology/radionuclide imaging ; Child ; Diagnosis, Differential ; Humans ; Male ; Neuroblastoma/*diagnosis/pathology ; Rhabdomyosarcoma/*diagnosis/pathology ; Sarcoma, Ewing/*diagnosis/pathology ; Tomography, X-Ray Computed

OBJECTIVETo observe the common characteristics of pathological morphology and immunohistochemical staining of olfactory neuroblastoma (ONB), and to raise the diagnostic ability for ONB.

METHODS34 cases of ONB, 11 cases of rhabdomyosarcoma (RMS) and 76 cases of malignant lymphoma (ML) were collected, the clinical information were investigated, and the biopsy samples were stained and observed as follows: (1) Routine HE staining and viewed under the light microscope. (2) Expression of neuron-specific enolase (NSE), chromogranin-A (CgA), leukocyte common antigen (LCA), desmin, and sarcometic actin (S-actin) were determined in both ONB and RMS cases. In addition, S-100 protein, cytokeratin (AE1/AE3) were detected in the ONB and myoglobulin detected in the RMS. ML samples were stained for LCA, CD45RO, CD56, and CD20. The NSE, CgA, desmin and S-actin were stained in 10 cases of NK/T cell ML and 9 cases of B cell ML additionally. (3) 4 cases of ONB, RMS and ML were observed under transmission electron microscope respectively.

RESULTSThe ages and clinical manifestations of ONB, RMS and ML were similar. The morphological characteristics of ONB included epithelial nests; net of angioma-like fibrous connective tissues; small round cells and small short spindle cells and nucleus; glandular and squamous liked epithelium; Homer-Wright and Flexner rosette; bunch of neurofibrilla, etc. NSE and CgA were expressed in small cells. S-100 protein was positive in the area of bunch of neurofibrilla. AE1/AE3 was positive in epithelial cells, LCA, while desmin and S-actin were all negative. Ultrastructurelly, there were neurosecretory granules and neurofibrilla in the cytoplasm of a few tumor cells. Although there were some similarities among ONB, RMS and ML under the light microscope, their characteristics of pathologic morphology, immunohistochemical staining and transmission electron microscope were different.

CONCLUSIONSThe features of the morphological changes are the most important basis to make diagnosis for ONB. The results of immunohistochemical staining can verify it further and play an important role in its differential diagnosis. Transmission electron microscope is very important but not essential for its diagnosis.

Adolescent ; Adult ; Aged ; Child ; Diagnosis, Differential ; Esthesioneuroblastoma, Olfactory ; diagnosis ; pathology ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; pathology ; Rhabdomyosarcoma ; diagnosis ; pathology

Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.
Child ; Choledochal Cyst ; Common Bile Duct/pathology* ; Diagnosis, Differential ; Humans ; Jaundice, Obstructive/etiology* ; Male ; Rhabdomyosarcoma, Embryonal/diagnosis*

OBJECTIVETo study the clinicopathologic characteristics of sclerosing rhabdomyosarcoma (SRMS) and its distinction from embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma (ARMS).

METHODSThe clinical, histologic and immunohistochemical features of 4 cases of SRMS were studied. The literature was reviewed.

RESULTSAll the 4 cases occurred in adults. The age of patients ranged from 20 to 54 years (mean = 41.5 years). The male-to-female ratio was 1:1. The tumor was located in the left wrist, right thigh, right face and right cheek respectively and the tumor size varied from 2.5 cm to 10 cm in dimension (mean = 5.7 cm). Histologically, SRMS was characterized by the presence of large amounts of heavily hyalinized matrix, mimicking osteoid or chondroid tissue. The tumor cells were composed predominantly of primitive small round cells which were arranged in diverse growth patterns, including fascicular, cord-like, single-file, trabecular, microalveolar and pseudovascular structures. A few rhabdomyoblasts were identified in 1 case. A second spindle cell component was focally found in 2 cases, resembling spindle cell rhabdomyosarcoma or peripheral nerve sheath tumor. Immunohistochemically, all cases showed diffuse staining for Myo D1 and focal staining for desmin. The staining for myogenin was often negative. Three of the cases also expressed muscle-specific actin and 2 cases were positive for alpha-smooth muscle actin. They were all negative for h-caldesmon, S-100 protein, CD31, CD34, AE1/AE3 and anaplastic lymphoma kinase protein.

CONCLUSIONSSRMS differs from ERMS and ARMS morphologically. Recent cytogenetic studies however suggest a histogenetic relationship with ERMS. Familiarity with its morphologic features and immunophenotype may help to distinguish this peculiar variant of rhabdomyosarcoma from a variety of lesions with abundant sclerosing matrix.

Actins ; metabolism ; Adult ; Chondrosarcoma ; pathology ; Combined Modality Therapy ; Desmin ; metabolism ; Diagnosis, Differential ; Facial Neoplasms ; metabolism ; pathology ; therapy ; Female ; Follow-Up Studies ; Hemangiosarcoma ; pathology ; Humans ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Osteosarcoma ; pathology ; Rhabdomyosarcoma ; classification ; metabolism ; pathology ; therapy ; Rhabdomyosarcoma, Alveolar ; classification ; pathology ; Rhabdomyosarcoma, Embryonal ; classification ; pathology ; Sclerosis ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; therapy ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo investigate the appearance and clinical value of MRI in the diagnosis of embryonal rhabdomyosarcoma in nasal cavity and paranasal sinuses.

METHODSThe clinical, pathological and MRI findings of five patients with pathology proved embryonal rhabdomyosarcoma in nasal cavity and paranasal sinuses were retrospectively analyzed.

RESULTSThe major clinical symptoms included nasal obstruction (4 cases), exophthalmus (4 cases), decreased eyesight (2 cases), epistaxis (1 case), decreased olfactory sensation (1 case) and restriction of eyeball movement (1 case). All 5 cases involved multi-location. Among them, 4 cases mainly located in the ethmoid sinus, one mainly located in the nasal septum. According to the IRS, 1 case was at stage II, the other 4 cases were at stage III. Compared to the grey matters, on T1 weighted image, the masses were homogenously isointensity in 2 cases, isointensity with patchy hyperintensity in 3 cases. On T2 weighted images, the masses were slightly hyperintensity with patchy hypointensity in 2 cases. Slightly hyperintensity with patchy hyperintensity in 3 cases. All 5 cases were markley heterogeneously enhanced after administration of contrast agents, with patchy of non-enhanced area. Of them, two were grape-like enhanced. Four cases presented with intratumor hemorrhage. Five with bony destruction. All of the 5 cases showed orbits and anterior cranial fossa meningeal involvement.

CONCLUSIONSThere are some special MRI findings in embryonal rhabdomyosarcoma located in nasal cavity and paranasal sinuses. MRI can depict the encroachment of the tumor accurately, and may play an important role in clinical staging and in curative effect evaluation.

Adolescent ; Adult ; Child ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Nasal Cavity ; Paranasal Sinus Neoplasms ; diagnosis ; pathology ; Retrospective Studies ; Rhabdomyosarcoma, Embryonal ; diagnosis ; pathology ; Young Adult