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15 February 2023 - Case of the Month #524

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Thanks to Dr. Kelly Magliocca, Emory University School of Medicine, Atlanta, Georgia, USA for contributing this case and discussion and to Dr. Molly Housley Smith, Pathology & Cytology Labs, Lexington, Kentucky, USA for reviewing the discussion.






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Case of the Month #524

Clinical history:
A 3 week old female infant had a pedunculated lesion of the anterior maxillary alveolar process, which was excised.

Histopathology images:


What is your diagnosis?

Click here for diagnosis, test question and discussion:



Diagnosis: Congenital granular cell epulis (congenital epulis of the newborn)

Test question (answer at the end):
Which of the following is a feature of congenital granular cell epulis?

A. Clinical presentation at ages 5-15
B. Detection within first trimester
C. Female predilection
D. Local recurrence after excision
E. Pseudoepitheliomatous hyperplasia



Discussion:
Congenital granular cell epulis (CGCE) is a benign lesion occurring on the alveolar ridge of the newborn. The condition is rare, with an estimated incidence of 0.0006% (Int J Otolaryngol 2009;2009:508780).

The characteristic presentation of CGCE is the presence of a solitary, soft pedunculated mass on the anterior maxillary alveolar ridge in a female infant. The female to male ratio is striking at nearly 8:1 (J Neonatal Surg 2015;4:17). While the maxillary anterior alveolar process is most commonly involved, reports of mandibular anterior alveolar process involvement and occasionally oral tongue localization have been reported (Ann Diagn Pathol 2011;15:157). Lesion size ranges from < 1 cm to 2 cm. Larger lesions may interfere with feeding. In some cases, a lesion can be identified in utero via ultrasonography within the third trimester (J Neonatal Surg 2015;4:17).

The etiology of CGCE remains unknown. A degenerative metabolic process affecting mesenchymal stem cells is speculated as pathogenesis (Virchows Arch 2009;454:303).

CGCE consists of sheet-like growth of polygonal cells with granular cytoplasm and well delineated cell membranes. Tumor nuclei are eccentric with inconspicuous nucleoli. Numerous delicate vascular channels are distributed throughout the lesion. Pseudoepitheliomatous hyperplasia of the overlying squamous epithelium is not a feature of CGCE, rather the overlying epithelium is attenuated and is without rete ridge formation. The stromal granular cells are positive for vimentin (not required for diagnosis), and negative for S100. In view of the characteristic population and histologic appearance, the differential diagnosis is relatively narrow, requiring distinction from granular cell tumor (SOX10+, S100+).

In general, treatment most commonly includes conservative excision. Recurrence is not reported. Some smaller lesions may spontaneously regress after birth.

Test question answer:
C. Female predilection. The characteristic presentation of CGCE is the presence of a solitary, soft pedunculated mass on the anterior maxillary alveolar ridge in a female infant. The female to male ratio is striking at nearly 8:1 (J Neonatal Surg 2015;4:17). Pseudoepitheliomatous hyperplasia is not present (the overlying epithelium is attenuated) and recurrence has not been reported.


Image 01 Image 02