Intraoral juvenile xanthogranuloma: an unusual presentation in an adult subject and literature review
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1
Fondazione Policlinico Universitario A. Gemelli, Università Cattolica del Sacro Cuore, Head and Neck Department, Italy
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2
Università Cattolica del Sacro Cuore, Department of Histopathology and Cytopathology, Italy
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3
Second University of Naples, Multidisciplinary Department of Medical-Surgical and Odontostomatological Specialties, Italy
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4
Università degli Studi di Bari, Interdisciplinary Department of Medicine, Italy
Aim.
The purpose of this case presentation is to describe an unusual case of intraoral juvenile xanthogranuloma (JXG), arising in a 36 years old Italian woman. Pertinent literature regarding the previously published intraoral JXGs is also reviewed.
Materials and Methods
A 36 years old Italian woman was referred to our Department for evaluation of a palate swelling arisen one month earlier. The patient’s medical history reported a breast cancer (diagnosis in 2016), treated with quadrantectomy, chemo and radiotherapy. Oral examination revealed the presence of a solitary, sessile, soft, light red, smooth-surfaced lesion. The lesion, which was located in the soft palate and measured approximately 5mm in diameter, had a clinical benign feature, nevertheless biopsy was mandatory in order to exclude the malignant nature. The lesion was surgically removed and the healing was uneventful.
Results.
The histological examination revealed normal oral epithelium with epitheliomorphic elements spread in the underlying connective tissue. Due to the clinical history of the woman, immunohistochemical staining was also performed to exclude a breast cancer metastasis. Immunohistochemistry showed positivity for cytoplasmic immunoreaction to CD68 and negativity for pan-citokeratins. Finally, a diagnosis of mononuclear variant of juvenile xanthogranuloma was made. At 6 months clinical follow-up, no signs of recurrence were detected.
Discussion.
Histiocytoses diseases encompass a group of proliferative disorders characterized by accumulation of dendritic and monocyte-derived cells in various tissues and organs; both children and adults can be affected. JXG is a benign, self-healing histiocytic disorder characterized by non-Langerhans cell histiocytes, which arises predominantly in infants and children skin with red or yellow asymptomatic papules and/or nodules. Its real incidence and the etiology are still unknown, however, the increased intracellular cholesterol biosynthesis and the uptake of low-density lipoprotein cholesterol might play a role in the pathogenesis. JXG have predominantly a cutaneous manifestation, nevertheless few cases of oral mucous membranes involvement had been reported in literature. Clinically, oral JXG appears like a well-defined, red to yellow, soft, solitary swelling, 2 to 15 mm in diameter, that may ulcerate and bleed, and affect especially the tongue and the hard palate. Histologically, classical hallmarks of JXG are the foamy cells and Touton giant cells. However, in our case, features of mononuclear variant of oral JXG were present, characterized by vacuolar foamy histocytes in absence of giant cells. Differential diagnoses may include dental abscess, fibroepithelial polyp, mucocele, vascular malformation, lipoma, gingival cyst, peripheral giant cell granuloma, foreign body granuloma, pyogenic granuloma and mucoepidermoid carcinoma. Moreover, the mononuclear JXG, may pathologically resemble a Langerhans cells histiocytosis, melanoma or balloon cell nevus.
Being a rare oral disease and resembling clinically and histologically many different lesions, the diagnosis of oral JXG may be challenging and often requires adjuvant immunohistochemical staining.
References
1. Le Boit PE, Burg G, Weedon D, et al. WHO Classification of Tumours: Pathology and Genetics Skin Tumours. Lyon: IARC Press; 2006.
2. Sánchez-Romero C, Cuenca Arriaga AI, Paes de Almeida O, Gutiérrez Cortés E. Oral juvenile xanthogranuloma in a child: Clinical, histological and immunohistochemical profile of a rare entity. J Cutan Pathol. 2018 Jul;45(7):515-521.
Keywords:
Juvenile xanthogranuloma,
Non-Langerhans cell histiocytosis,
Intraoral,
Palatal swelling,
Oral diseases
Conference:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine., Ancona, Italy, 19 Oct - 20 Oct, 2018.
Presentation Type:
Poster Presentation
Topic:
Oral Diseases
Citation:
Gioco
G,
Petrone
G,
Contaldo
M,
Lucchese
A,
Petruzzi
M and
Lajolo
C
(2019). Intraoral juvenile xanthogranuloma: an unusual presentation in an adult subject and literature review.
Front. Physiol.
Conference Abstract:
5th National and 1st International Symposium of Italian Society of Oral Pathology and Medicine..
doi: 10.3389/conf.fphys.2019.27.00059
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Received:
04 Nov 2018;
Published Online:
09 Dec 2019.
*
Correspondence:
Dr. Gioele Gioco, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica del Sacro Cuore, Head and Neck Department, Rome, Lazio, 00168, Italy, gioele.gioco@hotmail.it