Elizabeth Philipone

Mandibular embryonal rhabdomyosarcoma with cartilaginous metaplasia: report of a case and review of literature

Rhabdomyosarcoma (RMS) is a malignant tumor of skeletal muscle origin and frequently involves the head and neck region. It represents the second most common pediatric soft tissue sarcoma and accounts for 3% of all childhood cancers. Here, we report a case of embryonal RMS presenting as a right-sided facial swelling in a 7-year-old boy. Histologically, the tumor consisted of classic embryonal rhabdosarcomatous areas with metaplastic cartilage, in both initial biopsy and final resection specimens. Cartilaginous metaplasia arising in the background of RMS is a rare finding, thus raising a diagnostic challenge. To the best of our knowledge, this represents the first case of RMS with cartilaginous metaplasia in the oral cavity.

Renal cell carcinoma metastatic to the maxillary gingiva: A case report and review of the literature

Tumor metastasis to the oral cavity is rare and is usually an indication of late-stage disease and poor prognosis. While, there are reports of renal cell carcinoma (RCC) metastatic to oral cavity, vast majority of them are to the jaw. Herein, we present a case of a 78-year-old woman with RCC metastasis limited to the oral soft tissue without any bone involvement. As the lesion solely involved maxillary gingiva, it clinically mimicked that of a pyogenic granuloma, which is a reactive, nonneoplastic condition. This case was further complicated as the patient was unaware of primary cancer and appeared to be in good physical health. Her oral metastasis marked the initial manifestation of an otherwise silent primary renal cancer.

Mucosal Manifestations of Immunosuppression

The clinical presentation of oral lesions in immunosuppressed children is highly variable. In some cases, it is the oral manifestations that results in the discovery an underlying disease that is causing the immunosuppression. Immunosuppression/deficiency can either be primary or acquired. The World Health Organization recognizes more than 100 primary immune deficiency diseases. Examples include DiGeorge syndrome, complement deficiencies, gammaglobulinemia X-linked (Brutons) disease, immunoglobulin heavy chain deficiency, selective IgA deficiency, transient hypo-gammaglobulinemia of infancy, phagocytic disorders, severe congenital neutropenia (Kostmann syndrome), cyclic neutropenia, leukocyte adhesion defects, Chediak–Higashi syndrome, etc. Examples of acquired immunosuppression include HIV/AIDs and medication-induced immunosuppression seen in transplant patients as well as children undergoing chemotherapy.

Mucosal Manifestations of Nutritional Deficiencies

The cause of nutritional deficiencies is either from decreased intake (i.e., a diet that lacks essential nutrients) or the inability to absorb the nutrients (i.e., gastrointestinal conditions such as celiac disease). Children are more at risk for serious complications due to nutritional deficiencies than adults. Nutritional deficiencies affect children of all socioeconomic backgrounds but are more frequently seen in children from economically disadvantaged families and in children with underlying systemic diseases.

Mucosal Lumps and Bumps with Smooth Surfaces

Mucosal lumps and bumps are more accurately referred to as submucosal nodules. In general, submucosal nodules do not spontaneously resolve and often require surgical excision for treatment. A retrospective analysis of 3,129 oral pathology biopsy specimens from pediatric patients (0–18 years of age) submitted for diagnosis to Columbia University Medical Center, Department of Oral and Maxillofacial Pathology found mucocele and fibroma to comprise the two most frequently diagnosed soft tissue lesions. In this chapter, we review the clinical appearance, etiology, differential diagnosis, and recommended treatment for submucosal nodules.

White and Red Macules and Patches

It is not uncommon to encounter various white or red macules or patches in pediatric patients. These lesions are often transient and of various origins such as thermal insult, trauma, or frictional irritation. It is important to distinguish these lesions from true leukoplakias and erythroplakias which are by definition premalignant lesions. Fortunately true leuko- and erythroplakias are rare in children and adolescents.

Oral Soft Tissue Manifestations of Hematologic Abnormalities and Diseases

Hematologic disorders may exhibit nonspecific as well as pathognomonic oral manifestations.

Mucosal Manifestations of Gastrointestinal Disease

Gastrointestinal (GI) disorders – including celiac disease and irritable bowel syndrome (IBS), such as ulcerative colitis and Crohn’s disease – can present with intra-oral manifestations. Oral manifestations of GI disease are more common in children compared to adults. In some cases, particularly with Crohn’s disease, the oral lesions can present in the setting of subclinical GI symptoms or can even present several months to years before any GI manifestations. Oral lesions are the primary presenting sign preceding gastrointestinal symptoms in as much as 60 % of patients with Crohn’s disease.

Oral warty dyskeratoma of the retromolar trigone: An unusual presentation of a rare lesion

SCC: squamous cell carcinoma WD: warty dyskeratoma INTRODUCTION Warty dyskeratoma (WD), also known as isolated Darier disease or focal acantholytic dyskeratosis, is a rare mucocutaneous lesion. It usually presents as a solitary, asymptomatic umbilicated papule, most often in the head and neck region of middleto older-age adults. A slight male predilection has been observed. When it occurs in the oral cavity, WD is most commonly seen on the hard palate and alveolar ridge. Fewer than 50 cases of oral warty dyskeratoma are reported. Here we present a case of WD occurring on the left retromolar trigone in a 78-yearold man.