COM April 2012 Diagnosis

Large Ulcerated Swelling, Right Retromolar Pad & Tuberosity Area

Can you make the correct diagnosis?

This is a 15-year-old male who was referred for third molar removal to accommodate arch length deficiency.

1. TUGSE

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The oral cavity is frequently afflicted with surface ulcerations and for that reason, it is very important that clinicians view oral ulceration with some attention to etiology. The diffuse and deep nature of this ulceration does not preclude it from being traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) given the history of previous surgery in the area which may have been the source of trauma or trauma could have been factitious developed while the area was anaesthetized. The histology, however, was not supportive of TUGSE.

Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) ulcers are also known by other names, including eosinophilic ulcer, ulcerative eosinophilic granuloma of the tongue (which is not related to eosinophilic granuloma of the bone as in Langerhans Cell Histiocytosis), and traumatic granuloma. Traumatic ulcerative granuloma with stromal eosinophilia commonly occurs on the dorsal and lateral tongue, usually in patients with a history of trauma. The latter is not always clearly demonstrated. Although the tongue is the most common location, TUGSE has also been described in other areas such as the lips, buccal mucosa, gingiva and vestibule. TUGSE affects patients of all ages but is more common in adult males. It is described in children as young as infants with Riga-Fede, a disease that occurs during the first year of life as a result of chronic trauma in the sublingual area and ventral tongue occurring during breastfeeding due to the sharp-edged central lower incisors. The clinical presentation of TUGSE ranges from a small ulcer with rolled borders to an exophytic large lesion simulating a pyogenic granuloma. It can also present as a very large, indurated and deep ulcer simulating a squamous cell carcinoma or any other malignancy of the mouth. The duration of the non-healing ulcer can range from two weeks to many months. TUGSE tends to occur more in males than females. Histologically, these ulcers are deep lesions involving the underlying muscle, which may explain the process of slow healing and the tissue eosinophilia. Healing may take up to eight weeks, sometimes more.

2. Pyogenic Granuloma

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This ulcerated swelling is mostly in the retromolar pad area and away from the gingiva. It is, however, an ulcerated red mass and is focally exophytic. These characteristics are suggestive of a reactive process such as a pyogenic granuloma. However, the histology is not supportive of PG.

Pyogenic granuloma constitutes 85% of all reactive gingival swellings but can occur in areas other than the gingiva. It represents a profuse mass of vascular granulation tissue. It can be induced by trauma and local irritants such as excessive plaque, sharp fillings and dental calculus; it sometimes forms in an extraction socket in response to an irritant left in the socket. It can occur anywhere in the oral cavity and skin, especially the tongue, lips, fingers and nail beds. In the mouth, it occurs most commonly in the gingiva, especially the maxillary buccal and interproximal gingiva. Occasionally, it may surround the tooth. It is usually highly vascular, fast-growing, exophytic, lobular, sessile, and ulcerated or covered by pseudomembrane. The color changes from red to pink when it starts to heal. It occurs at any age and sex with a slight predilection for young females; it affects 1% of pregnant females. Pyogenic granuloma is usually painless except during eating, when bleeding and pain is described. Histologically, it presents as a mass of loose and vascular granulation tissue, usually with ulcerated or eroded surface epithelium and many inflammatory cells. A range of treatment modalities are available, including excision with removal of the local irritant, laser surgery, or intralesional injection with absolute alcohol, steroids or botulinum toxin. Scaling and polishing prior to surgical removal helps shrink the lesion. The prognosis is good, although recurrence is possible, especially during pregnancy.

3. Infectious Disease Ulcer

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Some infections, especially the deep granulomatous type of infectious diseases, may present in the oral cavity as diffuse, deep ulcers with rolled borders and at times as exophytic and ulcerated lesions. Because the retromolar area is not a good location for such lesions, the clinical presentation of persistent ulcer warrants that it be ruled out. Infectious disease-type ulcers of the mouth are more prevalent in patients who are immune compromised, which this patient is not. The histology is not supportive of any infectious-type disease.

The oral cavity is a site where a number of acute and chronic infectious diseases occur as a local primary disease or as a manifestation of a systemic disease. They include diseases of viral, fungal and bacterial origin. They usually present in multiples or in a diffuse manner ranging from ulcers to small nodular lesions to verrucoid lesions. Occasionally, granulomatous-type infectious diseases present as diffuse and deeply ulcerative lesions with rolled borders, simulating oral malignancies. These cases include ulceration induced by mycobacterium tuberculosis, deep fungal infections such as blastomycosis and histoplasmosis, and chancre of primary syphilis. Oral manifestations of tuberculosis and histoplasmosis are unusual without lung involvement and a disseminated disease; they occur more often in immune-compromised patients. The gingiva and the mandibular vestibule are more common locations, but can occur in other areas. Chancre can occur in any location, including the retromolar area, tongue, lips and hard palate.

4. Rhabdomyosarcoma

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Given the age of this patient and the resistance to healing, malignant neoplasms have to be considered and rhabdomysarcoma would be high on the list since it is common in the head and neck area and is common in children.

Rhabdomyosarcomas are malignant neoplasms of skeletal muscle origin constituting 4–8% of all malignancies in children under the age of 15. They most commonly occur in the head and neck (35%), followed by the genitourinary tract (23%) and the extremities (17%). In the head and neck area, the orbit is the most common location, followed by the nasal cavity, oropharynx and the oral cavity. Occurrences in the mouth account for 10–12% of all head and neck rhabdomyosarcomas. The tongue, palate and cheek are the most common sites in the oral cavity. Clinical presentations of rhabdomyosarcomas vary, ranging from small and exophytic to ulcerated, fungating and fast growing lesions, as is the case in this patient. Rhabdomyosarcomas can occasionally be associated with pain, trismus, paresthesia, facial palsy, and/or aural or nasal discharge. The variations in the clinical presentation can lead to delayed diagnosis. Three histologic types are described: embryonal, alveolar and pleomorphic. Embryonal and alveolar rhabdomyosarcoma are most common in children while pleomorphic rhabdomyosarcoma is most common in adults, though it is rare after the age of 45. They can also occur at birth; these cases, known as congenital rhabdomyosarcomas, are exceedingly rare. They are treated with multi-drug intensified chemotherapy combined with radiotherapy and surgery. This treatment modality has improved the overall 5-year survival rate for the pediatric group from 25% in 1970 to 74% currently. Rhabdomyosarcomas can metastasize, usually to the lungs, bone, and brain.

References

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  3. Hirshberg A, Amariglio N, Akrish S, Yahalom R, Rosenbaum H, Okon E, Kaplan I. Traumatic ulcerative granuloma with stromal eosinophilia: a reactive lesion of the oral mucosa. Am J Clin Pathol. 2006 Oct;126(4):522-9.
  4. Gharebaghi N, Monsouri SA, Darazam IA, Mansouri D, Sajadi MM, Mansouri N. A 40-year-old man with tongue lesions. Lingual and pulmonary tuberculosis (TB). Clin Infect Dis. 2011 May;52(10):1231, 1276-7.
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5. Mucoepidermoid Carcinoma

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It has been said that a swelling in the retromolar pad area that clinically looks like a mucocele should be suspected to be a mucoepidermoid carcinoma until histologically proven to be otherwise. This persistent ulcerated soft tissue swelling in the retromolar area raises the question of malignancy. However, the lesion does not look like a mucocele. The age of this patient and the location warrant that MEC be included on the differential diagnosis. The clinical presentation of a persistent ulcerated swelling adds to the concern. However, the histology is not supportive of MEC.

Mucoepidermoid carcinoma is a malignant neoplasm of salivary gland origin that usually presents as a smooth-surfaced swelling or a non-healing ulcer on the posterior and lateral hard and soft palate. Mucoepidermoid carcinoma is also occasionally described in the retromolar pad area. Three histologic types are reported: low, intermediate and high; the low-grade type is more common in the oral cavity. Mucoepidermoid carcinoma accounts for 10% of all salivary gland neoplasms. While the majority occurs in the parotid gland, some also occur in minor salivary glands, especially the palate, tongue, buccal mucosa, lips, and retromolar pad areas. It can occur at any age with a predilection for occurrence in young people. Studies by the Armed Forces Institute of Pathology (AFIP) find 44% of cases occurring in patients under 20 years of age, most commonly on the palate. Their youngest patient was nine months old. The low-grade lesions are slow-growing and painless, and not encapsulated; they sometimes resemble a mucocele, especially those at the retromolar pad area. Retromolar pad area mucoceles are rare, and for that reason it is best to biopsy early to exclude the possibility of a mucoepidermoid carcinoma masquerading as a mucocele. High-grade lesions tend to be more common in the parotid gland; they present as rapidly growing, painful lesions with facial nerve paralysis and sometimes with regional lymph node metastasis. Histologically, mucoepidermoid carcinoma consists of a variety of cell types and architectural patterns which constitute the three histologic gradings. Although low-grade mucoepidermoid carcinoma is characterized by an abundance of mucous-producing cells and duct-like structures with cystic dilation, the mere presence of certain types of cells and architecture should not be used to determine the histologic grade. Complete surgical removal with clean margins is the preferred treatment for the low-grade type. Radiotherapy has also been successfully used, especially when the tumor involves the surgical margins.

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