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CR0292 Paraneoplastic autoimmune multiorgan syndrome: 2 case reports
OOOO Volume 117, Number 5 Summary: A 10-year-old white boy presented for evaluation of an upper left gingival mass, which had been surgically removed and recurred twice. The lesion was hemorrhagic and caused delay in the eruption of the permanent canine. The patient’s medical history was otherwise noncontributory. Clinical examination found erythematous and swollen gingiva around the displaced left maxillary premolars along with hard tissue swelling in the area. A panoramic radiograph and a cone beam computed tomography scan found an irregular mixed radiolucent and radiopaque area in the left maxilla, accompanied by alveolar ridge erosion, cortical plate expansion, and displacement and divergence of the left premolars. A partial biopsy was performed, and histopathologic examination found scattered irregular tufts and lobules of variably sized vascular spaces inside the subepithelial connective tissue and between bone trabeculae. These thin-walled vascular channels were lined by densely packed spindle cells, exhibiting a “cannonball” appearance. Immunohistochemical evaluation found positivity for CD31, CD34, and smooth muscle actin. Based on these findings, a final diagnosis of intraosseous tufted angioma with bone erosion and soft tissue involvement was set. The lesion was surgically removed under general anesthesia; there were no signs of recurrence after 1 year of follow-up. Conclusions: Tufted angioma is a rare tumor of endothelial origin, almost exclusively affecting the soft tissues of the skin. Oral involvement is exceedingly rare. To the best of our knowledge, this is the first case of intraosseous tufted angioma located in the maxilla.
CR0391 LACRIMAL LYMPHOMA: AN UNUSUAL PRE¨ GREN SYNDROME Amanda Willis, SENTATION OF SJO L.H. Cheng, P.N. Plowman, A.S.M. Jawad, M. Bombardieri, A.R. Tappuni, Department of Oral Medicine, Institute of Dentistry, Barts and the London School of Medicine and Dentistry, London, United Kingdom Background: A 65-year-old woman was referred to the Dry Mouth Multidisciplinary Clinic at the Institute of Dentistry, Barts and the London School of Medicine and Dentistry, for investigation of suspected Sjögren syndrome after a diagnosis of marginal zone lacrimal lymphoma affecting her right lacrimal gland, which had been successfully treated with radiotherapy. Summary: She gave a history of at least 5 years of dry eyes and dry mouth. High-resolution salivary gland ultrasonography found atrophy in all major salivary glands, with changes consistent with established Sjögren syndrome. Blood investigations found positivity for Ro and La antibodies. A diagnosis of primary Sjögren syndrome was made, and she was treated accordingly. Approximately 6 months later at a routine review clinical appointment, she reported a 2-month history of a progressive but painless left-sided palatal swelling. Examination found a firm but spongy lesion extending from the first permanent molar tooth to the tuberosity, which did not cross the midline. The overlying epithelium was normal in appearance. Given her previous diagnosis of a lacrimal gland lymphoma, an urgent biopsy was performed and a diagnosis of marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT) type was confirmed on histology. After discussion by the multidisciplinary team, she is being worked up for further radiotherapy. Conclusions: Primary Sjögren syndrome presenting as a lymphoma of the lacrimal gland is rare and has been reported in the literature only once to our knowledge. Marginal zone lymphoma of the MALT type often presents in a multifocal fashion, and the risk for recurrence in other sites is in the region of 50% despite treatment of the primary site. We present a rare case of a
ABSTRACTS Abstracts e367 palatal lymphoma as a recurrence of a primary lacrimal gland lymphoma in a patient with primary Sjögren syndrome.
CR0292 PARANEOPLASTIC AUTOIMMUNE MULTIORGAN SYNDROME: 2 CASE REPORTS Sheila Galvin, Stephen Flint, Claire Healy, Mary Toner, Dublin Dental University Hospital Department: Department of Oral and Maxillofacial Surgery, Oral Medicine, Oral Pathology and Radiology, Dublin, Ireland Background: Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a heterogeneous autoimmune syndrome with multiple mucosal, cutaneous, and internal organ manifestations and a complex pathophysiology. Increasingly antibody-negative PAMS is being recognized, and we report 2 cases associated with non-Hodgkin lymphoma (NHL). Summary: Patient 1 is a 62-year-old man with a background of low-grade follicular NHL, twice treated with rituximab-based combination chemotherapy, who presented with extensive, somewhat lichenoid, oral ulceration and a lichenoid skin eruption. Initial oral biopsy found features of erythema multiforme, and a second found lichenoid features. Direct immunofluorescence (DIF), immunoblotting, immunoprecipitation, and indirect immunofluorescence (IIF) on multiple substrates were all negative. The oral ulceration was unresponsive to topical and intralesional steroids. Treatment has included prednisolone 0.75-1 mg/kg, intravenous immunoglobulin, and mycophenolate mofetil. His mouth is currently stable on 50 mg prednisolone daily, and his NHL is under observation. Patient 2 is a 59-year-old man with biopsy-proven oral lichen planus, previously well controlled with topical beclomethasone 0.5 mg mouthwash, who developed severe cheilitis and oral ulceration and a lichenoid skin eruption. Oral biopsy found features of severe erosive lichen planus and erythema multiforme. DIF was nonspecific, and IIF was negative on multiple substrates. There was no response to topical treatment and 6 weeks of 1 mg/kg oral prednisolone, so further investigations were carried out, including computed tomography (CT) of the thorax, abdomen, and pelvis, which found significant retroperitoneal thickening and lymphadenopathy. CT-guided biopsy confirmed diffuse large B cell lymphoma, and he has begun treatment. Conclusions: Up to 50% of patients with PAMS are antibody negative; postulated reasons include prior rituximab treatment, hypogammaglobulinemia, and cytotoxic T celledominant disease. These cases highlight the importance of clinical and histopathologic diagnosis with negative serology in this unusual condition, the treatment challenges in patients with ongoing malignancy, and the importance of searching for an underlying cause in patients who are unresponsive to therapy.
CR0436 PRIMARY ORAL TUBERCULOSIS Al Roshaidan Mohammed, Dharti N. Patel, Yagoub Alyami, Alexander R. Kerr, Sonal S. Shah, Department of Oral Pathology, Radiology and Medicine, New York University College of Dentistry, Brooklyn, NY, USA Background: Tuberculosis (TB) is a disease caused by Mycobacterium tuberculosis. It is primarily a pulmonary infection, although extrapulmonary infections are possible. Oral tuberculosis is rare and results from secondary exposure to infected sputum via ulcerations or minor masticatory trauma. It can be found on the tongue, palate, lips, buccal mucosa, and (less commonly) the gingiva, and it usually manifests as persistent ulceration.
CR0391 LACRIMAL LYMPHOMA: AN UNUSUAL PRE¨ GREN SYNDROME Amanda Willis, SENTATION OF SJO L.H. Cheng, P.N. Plowman, A.S.M. Jawad, M. Bombardieri, A.R. Tappuni, Department of Oral Medicine, Institute of Dentistry, Barts and the London School of Medicine and Dentistry, London, United Kingdom Background: A 65-year-old woman was referred to the Dry Mouth Multidisciplinary Clinic at the Institute of Dentistry, Barts and the London School of Medicine and Dentistry, for investigation of suspected Sjögren syndrome after a diagnosis of marginal zone lacrimal lymphoma affecting her right lacrimal gland, which had been successfully treated with radiotherapy. Summary: She gave a history of at least 5 years of dry eyes and dry mouth. High-resolution salivary gland ultrasonography found atrophy in all major salivary glands, with changes consistent with established Sjögren syndrome. Blood investigations found positivity for Ro and La antibodies. A diagnosis of primary Sjögren syndrome was made, and she was treated accordingly. Approximately 6 months later at a routine review clinical appointment, she reported a 2-month history of a progressive but painless left-sided palatal swelling. Examination found a firm but spongy lesion extending from the first permanent molar tooth to the tuberosity, which did not cross the midline. The overlying epithelium was normal in appearance. Given her previous diagnosis of a lacrimal gland lymphoma, an urgent biopsy was performed and a diagnosis of marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT) type was confirmed on histology. After discussion by the multidisciplinary team, she is being worked up for further radiotherapy. Conclusions: Primary Sjögren syndrome presenting as a lymphoma of the lacrimal gland is rare and has been reported in the literature only once to our knowledge. Marginal zone lymphoma of the MALT type often presents in a multifocal fashion, and the risk for recurrence in other sites is in the region of 50% despite treatment of the primary site. We present a rare case of a
ABSTRACTS Abstracts e367 palatal lymphoma as a recurrence of a primary lacrimal gland lymphoma in a patient with primary Sjögren syndrome.
CR0292 PARANEOPLASTIC AUTOIMMUNE MULTIORGAN SYNDROME: 2 CASE REPORTS Sheila Galvin, Stephen Flint, Claire Healy, Mary Toner, Dublin Dental University Hospital Department: Department of Oral and Maxillofacial Surgery, Oral Medicine, Oral Pathology and Radiology, Dublin, Ireland Background: Paraneoplastic autoimmune multiorgan syndrome (PAMS) is a heterogeneous autoimmune syndrome with multiple mucosal, cutaneous, and internal organ manifestations and a complex pathophysiology. Increasingly antibody-negative PAMS is being recognized, and we report 2 cases associated with non-Hodgkin lymphoma (NHL). Summary: Patient 1 is a 62-year-old man with a background of low-grade follicular NHL, twice treated with rituximab-based combination chemotherapy, who presented with extensive, somewhat lichenoid, oral ulceration and a lichenoid skin eruption. Initial oral biopsy found features of erythema multiforme, and a second found lichenoid features. Direct immunofluorescence (DIF), immunoblotting, immunoprecipitation, and indirect immunofluorescence (IIF) on multiple substrates were all negative. The oral ulceration was unresponsive to topical and intralesional steroids. Treatment has included prednisolone 0.75-1 mg/kg, intravenous immunoglobulin, and mycophenolate mofetil. His mouth is currently stable on 50 mg prednisolone daily, and his NHL is under observation. Patient 2 is a 59-year-old man with biopsy-proven oral lichen planus, previously well controlled with topical beclomethasone 0.5 mg mouthwash, who developed severe cheilitis and oral ulceration and a lichenoid skin eruption. Oral biopsy found features of severe erosive lichen planus and erythema multiforme. DIF was nonspecific, and IIF was negative on multiple substrates. There was no response to topical treatment and 6 weeks of 1 mg/kg oral prednisolone, so further investigations were carried out, including computed tomography (CT) of the thorax, abdomen, and pelvis, which found significant retroperitoneal thickening and lymphadenopathy. CT-guided biopsy confirmed diffuse large B cell lymphoma, and he has begun treatment. Conclusions: Up to 50% of patients with PAMS are antibody negative; postulated reasons include prior rituximab treatment, hypogammaglobulinemia, and cytotoxic T celledominant disease. These cases highlight the importance of clinical and histopathologic diagnosis with negative serology in this unusual condition, the treatment challenges in patients with ongoing malignancy, and the importance of searching for an underlying cause in patients who are unresponsive to therapy.
CR0436 PRIMARY ORAL TUBERCULOSIS Al Roshaidan Mohammed, Dharti N. Patel, Yagoub Alyami, Alexander R. Kerr, Sonal S. Shah, Department of Oral Pathology, Radiology and Medicine, New York University College of Dentistry, Brooklyn, NY, USA Background: Tuberculosis (TB) is a disease caused by Mycobacterium tuberculosis. It is primarily a pulmonary infection, although extrapulmonary infections are possible. Oral tuberculosis is rare and results from secondary exposure to infected sputum via ulcerations or minor masticatory trauma. It can be found on the tongue, palate, lips, buccal mucosa, and (less commonly) the gingiva, and it usually manifests as persistent ulceration.