OBJECTIVE: 10 summarize tne clinical features of the facial nerve tumors involving the internal auditory canal and promote the management of facial nerve tumor. METHOD: We retrospectively reviewed the clinical manifestations, the experiences of diagnosis and treatment of the facial nerve tumor involving the internal auditory canal. All these 5 cases were enrolled during January 2013 to Apr 2015. RESULT: Among the 5 cases, 3 cases were facial neurilemmoma and the others were facial neurofibroma. The main symptoms of facial nerve tumors involving the internal auditory canal most commonly were facial paralysis companied with hearing loss. All the patients accepted the surgical treatment with various approaches, 3 cases of translabyrinthine approach, 1 case of middle fossa approach, and 1 case of combination of translabyrinthine and transotic approach. Total tumor resection were achieved in all 5 cases. Facial-hypoglossal nerve anastomosis was performed in one case, another case was undergone great auricular nerve graft. CONCLUSION Surgical intervention for patients with facial neuroma involving internal auditory canal should be considered when facial weakness has deteriorated to grade 4. The management should be based on the patient's hearing, facial nerve function, tumor size and invasive extension to select the appropriate surgical procedures.
Anastomosis, Surgical ; Cranial Nerve Neoplasms ; diagnosis ; surgery ; Facial Nerve ; pathology ; surgery ; Facial Nerve Diseases ; diagnosis ; surgery ; Facial Paralysis ; complications ; Hearing Loss ; complications ; Humans ; Hypoglossal Nerve ; surgery ; Neurilemmoma ; diagnosis ; Neurofibroma ; diagnosis ; Retrospective Studies

Summary A 36 years old patient with hoarseness for 2 years and got worsen for one month, electronic laryngoscopy showed a red smooth-faced wide based neoplasm on the posterior 2/3 of the right side of the vocal cords. The neoplasm was excised under suspension laryngoscope . The pathologic results showed:Cells were weave patterned, infiltrative growth, mitotic figure was rare. Immunohistochemical results showed CD34 (-), SMA (-), DM (-), S - 100 (+). The pathological diagnosis was plexiform schwannoma.
Adult ; Hoarseness ; etiology ; Humans ; Laryngeal Neoplasms ; complications ; diagnosis ; surgery ; Laryngoscopes ; Laryngoscopy ; Neurilemmoma ; complications ; diagnosis ; surgery ; Syndrome ; Vocal Cords ; pathology

OBJECTIVETo investigate the clinical pathological features, diagnosis and differential diagnosis of pigmented dermatofibrosarcoma protuberance (PDFSP).

METHODSThe clinical history, histopathological features, immunohistochemical characteristics, treatment and prognosis were analyzed in seven cases of PDFSP. Fluorescence in situ hybridization (FISH) was used to detect the expression of COL1A1/PDGFB fusion gene, and related literature was reviewed.

RESULTSThe median age of the seven patients (4 females, 3 males) was 47 years with the tumors involving mostly the trunk (four cases). Histologically, PDFSP showed a cellular lesion composed of spindle cells arranged in short fascicles that form a distinct storiform pattern, and the pigmented bipolar or multipolar dendritic cells were present with tentacle like processes emanating from a nucleus containing zone. One case showed fibrosarcomatous change. The pigment was tinctorially similar to melanin. The spindle cells were positive for CD34 and vimentin, but negative for HMB45, Melan A, S-100, desmin, CD68 or α-SMA. HMB45, Melan A, S-100 and vimentin were expressed in the melanin containing cells in 4, 4, 5 and 7 cases, respectively. The labeling index of Ki-67 was 1%-8%. Among the 4 cases successfully examined by FISH, 3 showed t(17;22)(q21;q13) which suggested COL1A1/PDGFB fusion gene. Three patients were treated by wide local excision and four were treated by simple surgical excision. Two patients developed recurrences during the follow-up period of 12 to 123 months. Of those treated by wide local excision, none developed recurrence. No patient died in the follow-up period.

CONCLUSIONSPDFSP is a rare pigmented variant of DFSP and an intermediate grade malignant tumor. The orgin of the tumor cells is still controversial. Surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of PDFSP as there is a risk of misdiagonsing it as either pigmented tumors associated with neurocutaneous syndromes or a highly malignant melanocytic neoplasm.

Adult ; Aged ; Antigens, CD34 ; metabolism ; Child, Preschool ; Dermatofibrosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Neurilemmoma ; metabolism ; pathology ; Neurofibroma ; metabolism ; pathology ; Oncogene Proteins, Fusion ; metabolism ; Prognosis ; Retrospective Studies ; S100 Proteins ; metabolism ; Skin Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

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

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

Aged ; Angiomyolipoma ; metabolism ; pathology ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Leiomyoma ; metabolism ; pathology ; Lymphocytes ; pathology ; Male ; Nephrectomy ; Neurilemmoma ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

Primary gastric lymphoma is a rare gastric malignancy. Its diagnostic process is complex. Clinician may find initial diagnosis of primary gastric lymphoma unreliable, especially when it indicates the rarest subtype of gastric lymphoma, while its initial endoscopic presentation fails to raise the slightest suspicion of primary gastric lymphoma. A 53-year-old Korean man was diagnosed, by endoscopic examination, with a round submucosal tumor of the stomach. Deep endoscopic biopsy, however, confirmed CD5 positive gastric lymphoma. Surgical treatment was performed for diagnosis and treatment. Postoperative histological examination confirmed gastric schwannoma. Gastric schwannoma is a spindle cell tumor, characterized by a peripheral cuff-like lymphocytic infiltration. Deep endoscopic biopsy may have been misdirected to the peripheral lymphoid cuff, failing to acquire spindle cells. The literature has been reviewed, and options for diagnostic accuracy have been suggested.
Antigens, CD20/metabolism ; Antigens, CD5/metabolism ; Diagnosis, Differential ; Gastric Mucosa/metabolism/pathology ; Gastroscopy ; Humans ; Male ; Middle Aged ; Neurilemmoma/*diagnosis/pathology/surgery ; Stomach Neoplasms/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed

OBJECTIVETo study the clinical and pathologic features of gastric schwannomas.

METHODSThe macroscopic and microscopic features of 9 cases of gastric schwannoma were analyzed. Immunohistochemical study for S-100 protein, CD117, CD34, neurofilament, desmin, nestin, glial fibrillary acidic protein, platelet derived growth factor-alpha (PDGFR-α) and vimentin was carried out. Mutation analysis of c-kit gene (exon 9, 11, 13 and 17) and PDGFR-α gene (exon 12 and 18) in 1 case was examined by PCR amplification and direct sequencing.

RESULTSThe patients included 5 males and 4 females. The age of patients ranged from 42 to 81 years (median = 56.5 years). The size of the tumors ranged from 2 to 9 cm in greatest diameter. Follow-up data in 8 cases (from 1 month to 65 months) showed no evidence of recurrence or metastasis. Gross examination showed that gastric schwannomas were homogeneous, firm, yellow-white and bore no true fibrous capsule. Histologically, all cases were composed of fascicles of spindle cells associated with nuclear palisading, Verocay body formation and peripheral cuff of reactive lymphoid aggregates. Some of them showed degenerative changes including cyst formation, calcification, hemorrhage, necrosis and hyalinization. Immunohistochemical study showed that the tumor cells were strongly positive for S-100 protein and vimentin. There was various degree of staining for nestin (8/9) and glial fibrillary acidic protein (6/9). They were negative for CD117, CD34, neurofilament, desmin and smooth muscle actin. One case showed focal positivity for PDGFR-α (1/9), with no mutations found.

CONCLUSIONSGastric schwannomas share similar histologic features with conventional soft tissue schwannomas, in addition to the presence a reactive lymphoid cuff. The clinical, macroscopic, histologic and immunohistochemical features of gastric schwannomas were different from those of gastrointestinal stromal tumors and leiomyomas.

Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Exons ; Female ; Follow-Up Studies ; Gastrectomy ; methods ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Intermediate Filament Proteins ; metabolism ; Leiomyoma ; metabolism ; pathology ; Leiomyosarcoma ; metabolism ; pathology ; Male ; Middle Aged ; Mutation ; Nerve Tissue Proteins ; metabolism ; Nestin ; Neurilemmoma ; metabolism ; pathology ; surgery ; Neurofibroma ; metabolism ; pathology ; Receptor, Platelet-Derived Growth Factor alpha ; genetics ; metabolism ; S100 Proteins ; metabolism ; Stomach Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Primary tracheal tumours are rare and less frequently observed than bronchial tumours. Primary neurogenic tumours of the trachea as schwannomas or neurilemmomas are extremely uncommon. We report a tracheal schwannoma in a female patient who presented with breathlessness and wheeze, and she was being treated for asthma. Flexible bronchoscopy revealed a large pedunculated tracheal mass and biopsy confirmed schwannoma. She was treated with laser ablation with partial reduction of the tumour. Subsequently, she was lost to follow-up, although resection of the tumour with tracheal reconstruction was planned.
Adult ; Asthma ; diagnosis ; Biopsy ; Bronchoscopy ; Diagnosis, Differential ; Diagnostic Errors ; Dyspnea ; diagnosis ; etiology ; Female ; Follow-Up Studies ; Humans ; Neurilemmoma ; diagnosis ; surgery ; Radiography, Thoracic ; Reconstructive Surgical Procedures ; Respiratory Sounds ; diagnosis ; etiology ; Tomography, X-Ray Computed ; Trachea ; diagnostic imaging ; pathology ; surgery ; Tracheal Neoplasms ; diagnosis ; surgery

Adult ; Diagnosis, Differential ; Female ; Fibroma ; metabolism ; pathology ; Fingers ; Giant Cell Tumors ; metabolism ; pathology ; Humans ; Mucin-1 ; metabolism ; Nerve Sheath Neoplasms ; metabolism ; pathology ; surgery ; Neurilemmoma ; metabolism ; pathology ; Sclerosis ; metabolism ; pathology ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Tendons ; Young Adult