Extraskeletal Ewing's sarcoma is rarely found in the head and neck regions. We report an unusual case of extraskeletal Ewing's Sarcoma of the parapharynx region in a 49-year-old man who presented with blindness. MRI examination showed marked enhancement of tumor thrombosis involving the superior sagittal sinus, straight sinus, transverse sinus, sigmoid sinus, and internal jugular vein. The final diagnosis was extraskeletal Ewing's sarcoma after biopsy of the internal jugular vein thrombosis by histopathological evaluation and immunohistochemical assay. In addition, the patient was diagnosed as having adenocarcinoma of the rectum by biopsy of the rectal mass. The patient was treated with systemic chemotherapy and showed improved response with durable remission. The patient's visual acuity, however, did not improve.
Antineoplastic Agents/therapeutic use ; *Blindness ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Head and Neck Neoplasms/diagnosis/drug therapy/*pathology ; Humans ; Male ; Middle Aged ; Sarcoma, Ewing's/diagnosis/drug therapy/*pathology ; Vincristine/therapeutic use

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

Carcinoma ; drug therapy ; genetics ; metabolism ; pathology ; radiotherapy ; Desmoplastic Small Round Cell Tumor ; metabolism ; pathology ; Diagnosis, Differential ; Gene Rearrangement ; Head and Neck Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy ; Humans ; Keratin-20 ; metabolism ; Keratin-7 ; metabolism ; Lymphatic Metastasis ; Male ; Mediastinal Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy ; Melanoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; Nuclear Proteins ; genetics ; metabolism ; Oncogene Proteins ; genetics ; metabolism ; Oncogene Proteins, Fusion ; genetics ; metabolism ; Rhabdomyosarcoma ; metabolism ; pathology ; Thymus Neoplasms ; drug therapy ; genetics ; metabolism ; radiotherapy

BACKGROUND AND OBJECTIVEAmyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition.

METHODSClinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR).

RESULTSCase 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were found surrounding the amyloid deposits. Congo red staining showed positive of amyloid deposition in tumor tissues of both cases. Immunohistochemical staining revealed that LCA, CD19, CD20, CD79a and MUM1 expressions were positive in tumor cells, and Ki-67 expression was about 8%-10%. IgL restricted expression as kappa positive while lambda negative was found in both cases. PCR results showed monoclone gene rearrangement of IgH gene in both cases.

CONCLUSIONSOur findings suggest that amyloid deposition rarely present in both primary and metastatic tumors in PCMZL, and its diagnosis should be considered to avoid misdiagnosis. The patients with PCMZL should undergo regular examinations and chemotherapy as well as a long-term follow-up since it is apt to recur or relapse.

Aged ; Amyloidosis ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Antigens, CD ; metabolism ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Elbow ; Follow-Up Studies ; Head and Neck Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Interferon Regulatory Factors ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Prednisone ; therapeutic use ; Skin Neoplasms ; complications ; drug therapy ; metabolism ; pathology ; surgery ; Vincristine ; therapeutic use

Actins ; metabolism ; Diagnosis, Differential ; Granuloma, Plasma Cell ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; surgery ; Head and Neck Neoplasms ; diagnostic imaging ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Neoplasm Recurrence, Local ; Protein Kinase Inhibitors ; therapeutic use ; Pyrazoles ; therapeutic use ; Pyridines ; therapeutic use ; Tomography, X-Ray Computed ; Vimentin ; metabolism