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Epithelioid Hemangioendothelioma of the Maxilla in a Pediatric Patient, from Resection to Reconstruction: a Case Report
Poster Session
Figure 3 Specimen at 20X magnification with hematoxylin and eosin stain demonstrates the pedunculated nature of the osteochondroma with thick overlying proliferating cartilage cap and poorly organized cancellous bone. The endochondral ossification at the base of the cartilage cap is noted.
POSTER 38 Epithelioid Hemangioendothelioma of the Maxilla in a Pediatric Patient, from Resection to Reconstruction: a Case Report J. F. Williams: University of Mississippi Medical Center, L. Montague, R. Chandran Epithelioid Hemangioendothelioma (EH) is a rare, vascular neoplasm of the soft tissue first described by Weiss, et al in 1981. It has been described predominately in the soft tissue, but since its initial introduction it has been noted in numerous locations, including the lung, liver, viscera, and bone. The outcome of EH varies between sites; solitary and skin lesions appear relatively slowgrowing, whereas multi centric lesions, or those with histological atypia, and lesions from sites such as bone, liver, or lungs carry a higher rate of metastasis and have increased mortality. It is exceedingly rare in the head and neck region, with only approximately 30 documented cases from 1975 to 2014 to involve the oral cavity. Furthermore, only about 11 cases presented with a primary lesion involving the mandibular or maxillary gingiva. The World Health Organization describes EH as an intermediate malignant neoplasm due to its variability in clinical behavior. Due to its relative rarity, correct diagnosis can be a challenge. We present a case in which a 14-year old patient presented with an inflammatory, erythematous lesion of the right maxillary gingiva which was initially diagnosed as non-specific inflammation with an increased plasma cell component. Repeat review and expert consultation of the initial biopsy favored the diagnosis of EH which was treated by wide local excision. The resulting defect of the partial maxillectomy was an approximately 2cmx2cm bony defect that could be classified as a Brown type IIa defect. The patient also exe-394
hibited a persistent small oroantral fistula (OAF). Due to several factors and collaboration with the maxillofacial prosthetics department, the decision was made to reconstruct with zygomatic and dental implants with concomitant closure of the OAF via advancement of a right buccal fat pad. The decision to use a combination of zygoma and dental implants in this case was warranted due to advantages such as a shorter treatment time frame and cost reduction as well as decreased morbidity when compared to free bone grafting techniques in this young, healthy patient. The final stage of treatment was eventual delivery of a hybrid-RPD prosthesis to restore esthetics and function. Here, we will describe the clinical and histological features necessary for correct diagnosis of this rare entity, in addition to discussing treatment algorithms and ultimate decision making in developing a multi-disciplinery reconstructive plan to restore optimal oral health, esthetics, and function. References: 1. Salgarelli, Attilio, et al. Epithelioid hemangioendothelioma of the mandibular gingiva: A rare case of metastasis 4 years after radical excision and literature review. J Oral Maxillofac Pathol. 2016 Jan-Apr; 20(1): 137-141 2. Shabnum, Ali, et al. Epithelioid haemangioendothelioma of the mandibular gingiva: case report and literature review. Int J Surg Case Rep. 2015; 14: 194-198
POSTER 39 Varying Treatments for Osteomyelitis in Two Patients with Osteopetrosis Including the Use of Bone Morphogenetic Protein-2 S. N. Barnes: Vanderbilt University Medical Center, S. I. Lin, S. J. McKenna Osteopetrosis is a rare genetic condition affecting approximately 1:100,000- 500,000 persons.1 This disease is associated with a defect in osteoclast bone resorption.2 Osteomyelitis of the mandible is a common finding in the setting of osteopetrosis. Various treatment options have been described in the management of mandibular osteomyelitis in the setting of osteopetrosis. These treatments may include, alone or in combination, antibiotic administration, hyperbaric oxygen, debridement, and resection.3 Debridement, when necessary, may result in a critical mandibular defect without a suitable source of autogenous bone for grafting in the patient with osteopetrosis. To the best of our knowledge, the use of bone morphogenetic protein-2 (BMP-2) has not been described in the reconstruction of a mandibular defect in a patient with osteopetrosis. The purpose of this report is to describe, compare, and contrast two cases of osteopetrosis, as well as present the AAOMS 2017
Figure 3 Specimen at 20X magnification with hematoxylin and eosin stain demonstrates the pedunculated nature of the osteochondroma with thick overlying proliferating cartilage cap and poorly organized cancellous bone. The endochondral ossification at the base of the cartilage cap is noted.
POSTER 38 Epithelioid Hemangioendothelioma of the Maxilla in a Pediatric Patient, from Resection to Reconstruction: a Case Report J. F. Williams: University of Mississippi Medical Center, L. Montague, R. Chandran Epithelioid Hemangioendothelioma (EH) is a rare, vascular neoplasm of the soft tissue first described by Weiss, et al in 1981. It has been described predominately in the soft tissue, but since its initial introduction it has been noted in numerous locations, including the lung, liver, viscera, and bone. The outcome of EH varies between sites; solitary and skin lesions appear relatively slowgrowing, whereas multi centric lesions, or those with histological atypia, and lesions from sites such as bone, liver, or lungs carry a higher rate of metastasis and have increased mortality. It is exceedingly rare in the head and neck region, with only approximately 30 documented cases from 1975 to 2014 to involve the oral cavity. Furthermore, only about 11 cases presented with a primary lesion involving the mandibular or maxillary gingiva. The World Health Organization describes EH as an intermediate malignant neoplasm due to its variability in clinical behavior. Due to its relative rarity, correct diagnosis can be a challenge. We present a case in which a 14-year old patient presented with an inflammatory, erythematous lesion of the right maxillary gingiva which was initially diagnosed as non-specific inflammation with an increased plasma cell component. Repeat review and expert consultation of the initial biopsy favored the diagnosis of EH which was treated by wide local excision. The resulting defect of the partial maxillectomy was an approximately 2cmx2cm bony defect that could be classified as a Brown type IIa defect. The patient also exe-394
hibited a persistent small oroantral fistula (OAF). Due to several factors and collaboration with the maxillofacial prosthetics department, the decision was made to reconstruct with zygomatic and dental implants with concomitant closure of the OAF via advancement of a right buccal fat pad. The decision to use a combination of zygoma and dental implants in this case was warranted due to advantages such as a shorter treatment time frame and cost reduction as well as decreased morbidity when compared to free bone grafting techniques in this young, healthy patient. The final stage of treatment was eventual delivery of a hybrid-RPD prosthesis to restore esthetics and function. Here, we will describe the clinical and histological features necessary for correct diagnosis of this rare entity, in addition to discussing treatment algorithms and ultimate decision making in developing a multi-disciplinery reconstructive plan to restore optimal oral health, esthetics, and function. References: 1. Salgarelli, Attilio, et al. Epithelioid hemangioendothelioma of the mandibular gingiva: A rare case of metastasis 4 years after radical excision and literature review. J Oral Maxillofac Pathol. 2016 Jan-Apr; 20(1): 137-141 2. Shabnum, Ali, et al. Epithelioid haemangioendothelioma of the mandibular gingiva: case report and literature review. Int J Surg Case Rep. 2015; 14: 194-198
POSTER 39 Varying Treatments for Osteomyelitis in Two Patients with Osteopetrosis Including the Use of Bone Morphogenetic Protein-2 S. N. Barnes: Vanderbilt University Medical Center, S. I. Lin, S. J. McKenna Osteopetrosis is a rare genetic condition affecting approximately 1:100,000- 500,000 persons.1 This disease is associated with a defect in osteoclast bone resorption.2 Osteomyelitis of the mandible is a common finding in the setting of osteopetrosis. Various treatment options have been described in the management of mandibular osteomyelitis in the setting of osteopetrosis. These treatments may include, alone or in combination, antibiotic administration, hyperbaric oxygen, debridement, and resection.3 Debridement, when necessary, may result in a critical mandibular defect without a suitable source of autogenous bone for grafting in the patient with osteopetrosis. To the best of our knowledge, the use of bone morphogenetic protein-2 (BMP-2) has not been described in the reconstruction of a mandibular defect in a patient with osteopetrosis. The purpose of this report is to describe, compare, and contrast two cases of osteopetrosis, as well as present the AAOMS 2017