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

Schwannoma, which manifests as a smooth and solitary, encapsulated lesion, originates from the nerve sheaths of Schwann cells. Ancient schwannoma, a rare histologic variant of schwannoma, characterized by its distinctive degenerative changes. Basically, treatment entails complete removal of the mass, with maximal safeguarding of the nerve. However, depending on tumor size and extent, complete removal can be problematic. Le Fort I osteotomy in orthognathic surgery is widely utilized and can be effective to remove the skull base tumors. A 25-year-old woman presented with stabbing pain of 1-2 seconds duration occurring every two hours in the interior of the right face, which condition had persisted for one year. Magnetic resonance imaging showed mass lesion in the right retromaxillary area. Mass in retromaxillary area was removed by Le Fort I osteotomy. Pathologic examination confirmed the diagnosis of ancient schwannoma. In the course of a six-month follow-up, neither recurrence nor malocclusion was observed.
Adult ; Diagnosis ; Facial Pain ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Malocclusion ; Neurilemmoma* ; Orthognathic Surgery ; Osteotomy* ; Recurrence ; Schwann Cells ; Skull Base

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

Adenoma, Pleomorphic ; diagnosis ; diagnostic imaging ; surgery ; Adolescent ; Adult ; Aged ; Angiography, Digital Subtraction ; Carcinoma, Adenoid Cystic ; diagnosis ; diagnostic imaging ; surgery ; Chemotherapy, Adjuvant ; Child ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neurilemmoma ; diagnosis ; diagnostic imaging ; surgery ; Oral Surgical Procedures ; methods ; Postoperative Complications ; Radiotherapy, Adjuvant ; Tomography, X-Ray Computed ; Young Adult

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

Intracerebral schwannomas are rare and there have been none reported in Korea. We present the case of a 25-year-old man with newly developed right-side weakness and recent seizure aggravation. His seizures started approximately 9 years prior to admission. At that time, a 1 cm diameter intra-axial enhancing mass at the left precentral gyrus was found on magnetic resonance image (MRI). After 9 years of observation and treatment with antiepileptic medication, an MRI taken due to symptom aggravation revealed peri-tumoral cyst formation with tumor enlargement. The tumor was surgically removed. Subsequently, right-side weakness diminished and there was good seizure control. Pathologic diagnosis was schwannoma. Schwannoma is a very rare tumor and there are no pathognomonic findings on radiologic images; thus, it is challenging to make a correct diagnosis. However, considering the natural course and excellent prognosis after surgical treatment of this kind of intra-axial mass with benign features, early surgery for diagnosis and proper treatment is highly recommended.
Adult ; Diagnosis ; General Surgery ; Humans ; Korea ; Magnetics ; Magnets ; Neurilemmoma* ; Prognosis ; Seizures