Juvenile fibromatosis of the mandible mimicking a malignant neoplasm : a case report / Laurenceburg Rodriguez.

By:

Rodriguez, Laurenceburg, MD [author]

Language: English Publication details: Fairview, Quezon City Department of Otolaryngology, FEU-NRMF, 2010Description: 20 pages: photos (colored); (in folder)Content type:

text

Media type:

unmediated

Carrier type:

volume

LOC classification:

ENT20100002

Summary: ABSTRACT: This is a case of J.C, 5 year old male with a rapidly enlarging submandibular mass initially diagnosed as Submandibular New Growth probably malignant. Panoramic Xray showed bony erosions on the body of right mandible and CT scan showed large soft tissue density on the right paramandibular area. Fine Needle Aspiration Biopsy revealed inconclusive results while initial incision biopsy revealed Benign Fibrohistocytoma. Repeat incision biopsy revealed Spindle Cell Tumor, to consider Fibromatosis, cannot totally rule out Embryonal Rhabdomyosarcoma, spindle cell type, Immunohistochemical staining with desmin, vimentin, and S-100 was most compatible with Odontogenic Myxofibroma. The patient was subjected to chemotherapy with vinblastine and methotrexate. Due to progressively enlarging mass despite of chemotherapy, wide excision with segmental mandibulectomy of the right submandibular mass was done. Final histopathology revealed Spindle Cell Tumor, to consider Juvenille Fibromatosis, cannot totally rule out Rhabdomyosarcoma, The right and left mandibular borders were negative for tumor involvement. The final standpoint for the patient is Fibromatosis based on the following clinical presentation and histopathological analysis: proliferation of well differential fibroblast, infiltration pattern of growth, presence of variable amount of collagen proliferating cells, lack of cytologic features of malignancy, scanty or absent metastatic, aggressive clinical behavior but lack of capacity to metastasize distantly, and positive immunohistochemical staining with vimentin.

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Includes appendices and bibliographical references.

ABSTRACT: This is a case of J.C, 5 year old male with a rapidly enlarging submandibular mass initially diagnosed as Submandibular New Growth probably malignant. Panoramic Xray showed bony erosions on the body of right mandible and CT scan showed large soft tissue density on the right paramandibular area. Fine Needle Aspiration Biopsy revealed inconclusive results while initial incision biopsy revealed Benign Fibrohistocytoma. Repeat incision biopsy revealed Spindle Cell Tumor, to consider Fibromatosis, cannot totally rule out Embryonal Rhabdomyosarcoma, spindle cell type, Immunohistochemical staining with desmin, vimentin, and S-100 was most compatible with Odontogenic Myxofibroma. The patient was subjected to chemotherapy with vinblastine and methotrexate. Due to progressively enlarging mass despite of chemotherapy, wide excision with segmental mandibulectomy of the right submandibular mass was done. Final histopathology revealed Spindle Cell Tumor, to consider Juvenille Fibromatosis, cannot totally rule out Rhabdomyosarcoma, The right and left mandibular borders were negative for tumor involvement. The final standpoint for the patient is Fibromatosis based on the following clinical presentation and histopathological analysis: proliferation of well differential fibroblast, infiltration pattern of growth, presence of variable amount of collagen proliferating cells, lack of cytologic features of malignancy, scanty or absent metastatic, aggressive clinical behavior but lack of capacity to metastasize distantly, and positive immunohistochemical staining with vimentin.

Research - Department of Otolaryngology

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