- Email: [email protected]
Central mucoepidermoid carcinoma arising in an odontogenic cyst
296 Abstracts
sarcomatous pattern with spindled cells in a storiform configuration was seen in 3 tumors (27.3%). Mitoses were evident in 9 of 11 tumors, with 4 (36.4%) exhibiting mitoses in almost every medium power field. Atypical mitoses were seen in only 2 tumors. Clear cells were present in 6 tumors (54.5%), and 2 had pseudoglandular aggregates. Tumors of 3 of the 4 patients who died of disease had notable numbers of clear cells. Treatment included curettage with or without electrocautery, radiation, and resection. Patients who were seemingly cured were those with the fewest recurrences and those undergoing radical surgery early in the course of their disease. All treatments other than surgery were met with recurrence. The 7 patients who recovered from their disease had an average of 0.875 recurrences (range, 0-3 recurrences). The 4 patients who died with tumors averaged 6.25 recurrences (range 4-9). The average interval between recurrences was 16.32 months (range, 1-48 months).
CENTRAL MUCOEPIDERMOID CARCINOMA ARISING IN AN ODONTOGENIC CYST. G. Pringle, D. Loggi Jr, S.-Y. Chen, Temple University, Philadelphia, Pa, and Cape May Court House, NJ. Central mucoepidermoid carcinoma (MEC) is a rare neoplasm. It occurs most commonly in the posterior mandibles of middle-aged adults. The radiographic features of central MEC are shared by a number of central gnathic lesions and thus are considered nonspecific. Most cases are low-grade malignancies typically presenting with cortical expansion. High-grade lesions can produce bone perforation, paresthesia, nodal metastasis, local recurrence, and death. The hypothesized histogenetic origins of central MEC include ectopic enclaved salivary gland tissue and multipotential epithelial cells of odontogenic cysts. Many reported cases provide circumstantial evidence of an odontogenic epithelial origin, but few reports provide microscopic corroboration of such histogenesis. We report a case of a 79-year-old woman with a large, multilocular radiolucency extending across the midline of the anterior mandible. The lesion was initially diagnosed as glandular odontogenic cyst on the basis of incisional biopsy results. Tissue submitted after enucleation through peripheral ostectomy confirmed the diagnosis of glandular odontogenic cyst. However, cyst lining epithelium was focally thickened and composed of clear cells with a luminal layer of mucous cells. In adjacent areas, invading strands of cyst-lining epithelium and separate cystic islands of low-grade MEC composed of mucous cells, clear cells, and some epidermoid cells in the connective tissue wall were strongly suggestive of an odontogenic origin. The relationship between mucoepidermoid carcinoma and glandular odontogenic cyst is reviewed.
OSTEOSARCOMA EX FLORID OSSEOUS DYSPLASIA: REPORT OF A CASE. R. Melrose, and J. Handlers, Oral Pathology Associates, Inc, Los Angeles, Calif. History. A 36-year-old black woman presented to the Los Angeles County/USC General Hospital Dental Service with increasing pain and rapid swelling of the left face and jaw. Several weeks earlier, 2 mandibular molars had been extracted elsewhere because of pain, but her symptoms worsened. Marked swelling of the left face was observed. There was no evidence of local redness, heat, or drainage. Intraorally, marked nontender, lobular expansion of the left mandible was present. There was no paresthesia. Radiographs revealed a diffuse, ill-defined radiopacity with buccal and lingual expansion. Radiographs taken 3 years earlier (1997) disclosed bilateral radiopacities of the alveolar processes of the maxilla and mandible consistent with florid osseous dysplasia (FOD). There was no evidence of hypercementosis. A comparison of current films with a film of the affected area obtained in 1997 revealed a distinct change in a large
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY September 2003 lobular opacity of the left mandible. Biopsy was performed, and a diagnosis of high-grade osteosarcoma was made. Metastatic work-up was negative. A core biopsy specimen from the right mandible displayed microscopic changes compatible with cemento-osseous dysplasia. Resection was performed, and the diagnosis of osteosarcoma was confirmed. After discharge, the patient was lost to follow-up. Comment. This patient represents the second reported instance of sarcoma arising in FOD. A case of osteosarcoma arising in Paget’s disease of the mandible has recently been reported; there was microscopic evidence of cemento-osseous dysplasia adjacent to the tumor, but the authors concluded that the tumor arose in pagetic bone. It has now been established that, in addition to osteomyelitis, sarcomas can complicate the course of FOD. It seems prudent to recommend continuing follow-up for patients with FOD.
POSTIRRADIATION LEIOMYOSARCOMA OF THE MAXILLA: REPORT OF A CASE IN A PATIENT WITH PRIOR TREATMENT FOR RETINOBLASTOMA. P. Sedghizadeh, F. Angiero, C. Allen, Y. Rawal, E. Albright, The Ohio State University, Columbus. Postirradiation sarcoma is a well-defined entity; however, only a few case reports document such lesions in the head and neck region. A 30-year-old man presented for evaluation of a painful mass of the left posterior aspect of the maxilla. His medical history was significant for unilateral retinoblastoma of the eye that was diagnosed in infancy and treated with a combination of surgical exenteration of the eye and radiation therapy. A biopsy specimen taken from the maxillary mass revealed a spindle cell malignancy that, taken with immunohistochemical findings, was deemed most consistent with leiomyosarcoma. Further investigation revealed the patient to be the father of 3 children, each of whom developed unilateral retinoblastoma in infancy. The role of the retinoblastoma gene in the pathogenesis of retinoblastoma and postirradiation sarcoma is discussed.
LANGERHANS CELL HISTIOCYTOSIS OF THE HEAD AND NECK IN CHILDHOOD: AN IMMUNOCYTOCHEMICAL AND ULTRASTRUCTURAL STUDY. J. Hicks, C. Flaitz, E. Friedman, Texas Children’s Hospital, Baylor College of Medicine, University of Texas at Houston Dental Branch. Purpose. This study evaluated the clinicopathologic, immunocytochemical, and ultrastructural features of Langerhans cell histiocytosis (LCH) of the head and neck (H&N) region in children. Design. The anatomic pathology files spanning a 15-year period at Texas Children’s Hospital were searched for LCH cases. Demographic information, clinical history, paraffin tissue blocks, microscopic slides, and electron microscopic files were available for review. Immunocytochemistry for CD1a and CD207 (Langerin, type II transmembrane protein restricted to Langerhans’ cells) was performed on cases. One hundred thirty-four cases of LCH were identified, with 69 cases involving the H&N region. Results. The mean patient age at diagnosis was 3.9 years (range, 3 months–15 years), with the majority being younger than or at 4 years of age. The male-to-female ratio was 1.2:1.0. The H&N LCH sites were as follows: skull, 42%; skin, 22%; lymph nodes, 13%; orbit, 9%; auditory canal, 4%; mandible, 3%; gingiva, 3%; vertebra, 1%; thyroid, 1%; and not otherwise specified, 2%. CD1a and CD207 immunoreactivity occurred in 87% (60/69) and 91% (63/69) of patients with H&N LCH, respectively. Electron microscopy revealed pentalaminar structures in all specimens examined (42/42). Other electron microscopy findings included multivesicular bodies, tubuloreticular inclusions, and curvilinear membrane formations. Lami-
sarcomatous pattern with spindled cells in a storiform configuration was seen in 3 tumors (27.3%). Mitoses were evident in 9 of 11 tumors, with 4 (36.4%) exhibiting mitoses in almost every medium power field. Atypical mitoses were seen in only 2 tumors. Clear cells were present in 6 tumors (54.5%), and 2 had pseudoglandular aggregates. Tumors of 3 of the 4 patients who died of disease had notable numbers of clear cells. Treatment included curettage with or without electrocautery, radiation, and resection. Patients who were seemingly cured were those with the fewest recurrences and those undergoing radical surgery early in the course of their disease. All treatments other than surgery were met with recurrence. The 7 patients who recovered from their disease had an average of 0.875 recurrences (range, 0-3 recurrences). The 4 patients who died with tumors averaged 6.25 recurrences (range 4-9). The average interval between recurrences was 16.32 months (range, 1-48 months).
CENTRAL MUCOEPIDERMOID CARCINOMA ARISING IN AN ODONTOGENIC CYST. G. Pringle, D. Loggi Jr, S.-Y. Chen, Temple University, Philadelphia, Pa, and Cape May Court House, NJ. Central mucoepidermoid carcinoma (MEC) is a rare neoplasm. It occurs most commonly in the posterior mandibles of middle-aged adults. The radiographic features of central MEC are shared by a number of central gnathic lesions and thus are considered nonspecific. Most cases are low-grade malignancies typically presenting with cortical expansion. High-grade lesions can produce bone perforation, paresthesia, nodal metastasis, local recurrence, and death. The hypothesized histogenetic origins of central MEC include ectopic enclaved salivary gland tissue and multipotential epithelial cells of odontogenic cysts. Many reported cases provide circumstantial evidence of an odontogenic epithelial origin, but few reports provide microscopic corroboration of such histogenesis. We report a case of a 79-year-old woman with a large, multilocular radiolucency extending across the midline of the anterior mandible. The lesion was initially diagnosed as glandular odontogenic cyst on the basis of incisional biopsy results. Tissue submitted after enucleation through peripheral ostectomy confirmed the diagnosis of glandular odontogenic cyst. However, cyst lining epithelium was focally thickened and composed of clear cells with a luminal layer of mucous cells. In adjacent areas, invading strands of cyst-lining epithelium and separate cystic islands of low-grade MEC composed of mucous cells, clear cells, and some epidermoid cells in the connective tissue wall were strongly suggestive of an odontogenic origin. The relationship between mucoepidermoid carcinoma and glandular odontogenic cyst is reviewed.
OSTEOSARCOMA EX FLORID OSSEOUS DYSPLASIA: REPORT OF A CASE. R. Melrose, and J. Handlers, Oral Pathology Associates, Inc, Los Angeles, Calif. History. A 36-year-old black woman presented to the Los Angeles County/USC General Hospital Dental Service with increasing pain and rapid swelling of the left face and jaw. Several weeks earlier, 2 mandibular molars had been extracted elsewhere because of pain, but her symptoms worsened. Marked swelling of the left face was observed. There was no evidence of local redness, heat, or drainage. Intraorally, marked nontender, lobular expansion of the left mandible was present. There was no paresthesia. Radiographs revealed a diffuse, ill-defined radiopacity with buccal and lingual expansion. Radiographs taken 3 years earlier (1997) disclosed bilateral radiopacities of the alveolar processes of the maxilla and mandible consistent with florid osseous dysplasia (FOD). There was no evidence of hypercementosis. A comparison of current films with a film of the affected area obtained in 1997 revealed a distinct change in a large
ORAL SURGERY ORAL MEDICINE ORAL PATHOLOGY September 2003 lobular opacity of the left mandible. Biopsy was performed, and a diagnosis of high-grade osteosarcoma was made. Metastatic work-up was negative. A core biopsy specimen from the right mandible displayed microscopic changes compatible with cemento-osseous dysplasia. Resection was performed, and the diagnosis of osteosarcoma was confirmed. After discharge, the patient was lost to follow-up. Comment. This patient represents the second reported instance of sarcoma arising in FOD. A case of osteosarcoma arising in Paget’s disease of the mandible has recently been reported; there was microscopic evidence of cemento-osseous dysplasia adjacent to the tumor, but the authors concluded that the tumor arose in pagetic bone. It has now been established that, in addition to osteomyelitis, sarcomas can complicate the course of FOD. It seems prudent to recommend continuing follow-up for patients with FOD.
POSTIRRADIATION LEIOMYOSARCOMA OF THE MAXILLA: REPORT OF A CASE IN A PATIENT WITH PRIOR TREATMENT FOR RETINOBLASTOMA. P. Sedghizadeh, F. Angiero, C. Allen, Y. Rawal, E. Albright, The Ohio State University, Columbus. Postirradiation sarcoma is a well-defined entity; however, only a few case reports document such lesions in the head and neck region. A 30-year-old man presented for evaluation of a painful mass of the left posterior aspect of the maxilla. His medical history was significant for unilateral retinoblastoma of the eye that was diagnosed in infancy and treated with a combination of surgical exenteration of the eye and radiation therapy. A biopsy specimen taken from the maxillary mass revealed a spindle cell malignancy that, taken with immunohistochemical findings, was deemed most consistent with leiomyosarcoma. Further investigation revealed the patient to be the father of 3 children, each of whom developed unilateral retinoblastoma in infancy. The role of the retinoblastoma gene in the pathogenesis of retinoblastoma and postirradiation sarcoma is discussed.
LANGERHANS CELL HISTIOCYTOSIS OF THE HEAD AND NECK IN CHILDHOOD: AN IMMUNOCYTOCHEMICAL AND ULTRASTRUCTURAL STUDY. J. Hicks, C. Flaitz, E. Friedman, Texas Children’s Hospital, Baylor College of Medicine, University of Texas at Houston Dental Branch. Purpose. This study evaluated the clinicopathologic, immunocytochemical, and ultrastructural features of Langerhans cell histiocytosis (LCH) of the head and neck (H&N) region in children. Design. The anatomic pathology files spanning a 15-year period at Texas Children’s Hospital were searched for LCH cases. Demographic information, clinical history, paraffin tissue blocks, microscopic slides, and electron microscopic files were available for review. Immunocytochemistry for CD1a and CD207 (Langerin, type II transmembrane protein restricted to Langerhans’ cells) was performed on cases. One hundred thirty-four cases of LCH were identified, with 69 cases involving the H&N region. Results. The mean patient age at diagnosis was 3.9 years (range, 3 months–15 years), with the majority being younger than or at 4 years of age. The male-to-female ratio was 1.2:1.0. The H&N LCH sites were as follows: skull, 42%; skin, 22%; lymph nodes, 13%; orbit, 9%; auditory canal, 4%; mandible, 3%; gingiva, 3%; vertebra, 1%; thyroid, 1%; and not otherwise specified, 2%. CD1a and CD207 immunoreactivity occurred in 87% (60/69) and 91% (63/69) of patients with H&N LCH, respectively. Electron microscopy revealed pentalaminar structures in all specimens examined (42/42). Other electron microscopy findings included multivesicular bodies, tubuloreticular inclusions, and curvilinear membrane formations. Lami-