Congenital epulis of the newborn

Kumar, R Mahesh; Bavle, Radhika M; Umashankar, D N; Sharma, Ridhima

doi:10.4103/0973-029x.174642

Cited by 29 publications

(41 citation statements)

References 17 publications

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“…The lesions can vary from a few millimetres in diameter to large tumors obstructing the airway or interfering with feeding, which are treated with surgery. 12 Other rare differentials include rhabdomyosarcoma, fibrosarcoma, paraganglioma, and neuroblastoma metastasis.…”

Section: Discussionmentioning

confidence: 99%

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion

et al. 2019

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Alveolar soft part sarcoma (ASPS) is an exceptionally rare non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), characterized by the translocation t(X;17) p(11.2;q25). This translocation results in the chimeric ASPSCR1-TFE3 transcription factor which drives tumorigenesis. Complete surgical resection is crucial in allowing a successful outcome in these cases. Here, we describe an 11-month-old female infant who presented with a well-circumscribed lesion of the tongue, with the clinical and radiologic appearances of an infantile hemangioma. This led to an initial plan for surveillance management. However, the mass continued to enlarge and the lesion was therefore biopsied when the infant was 17 months old. Histology showed plump epithelioid tumor cells, in many places lining pseudoalveolar spaces. Occasional Pas-D inclusions were present in the cytoplasm. Immunostaining showed nuclear positivity for TFE-3. Real-time quantitative polymerase chain reaction testing confirmed the presence of ASPSCR1-TFE3 fusion transcripts, characteristic of the translocation t(X;17) p(11.2;q25) observed in ASPS. This represents the youngest reported ASPS case with a confirmed molecular diagnosis. Complete surgical resection was undertaken and a surveillance imaging schedule implemented. This case highlights the need for regular review of the initial diagnosis and the importance of multidisciplinary discussion and early biopsy where the clinical course does not follow that expected for the putative (nonhistologically confirmed) diagnosis.

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Section: Discussionmentioning

confidence: 99%

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion

et al. 2019

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“…In addition, it can also be seen on the tongue more rarely. [4,5] Clinical findings vary according to the size of the tumor. While small tumors have almost no signs, large tumors can cause respiratory and feeding problems.…”

Section: Discussionmentioning

confidence: 99%

“…Prenatal diagnosis is difficult because tumor generally develops beyond the 22nd week of gestation. [4] In majority, the prenatal diagnosis of CGCE were made in late gestation as noted in the index cases. They are mostly recognized at birth except in cases where the size of tumor is very small.…”

Section: Discussionmentioning

confidence: 99%

“…[2] It is characterized by single or occasionally multiple smooth surfaced sessile or pedunculated masses with a pink or reddish color, protruding through the oral cavity with a potential to disrupt feeding of the newborn and rarely causes respiratory distress. [3][4][5][6] Huge CGCE poses diagnostic challenges and must be differentiated with intraoral teratoma or hemangioma. The reasons for diagnostic difficulty are the rarity of the tumor and the low index of suspicion.…”

Section: Introductionmentioning

confidence: 99%

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Congenital granular cell epulis: A case series

2020

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Background: Congenital granular cell epulis (CGCE) is an extremely rare intraoral tumor of the newborn with a potential to disrupt feeding and produce respiratory distress. Case Series: We report two newborns presented with mass protruding off the mouth since birth. The mass was arising from alveolar ridge in both cases and dealt with surgical excision. Histopathology revealed it congenital granular cell epulis. Postoperative recovery and follow-up are uneventful. Conclusion: Congenital granular cell epulis is a benign lesion though not quite uncommon, may cause diagnostic challenges. Early excision and histopathology rule out malignant lesions.

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“…CE is seen in girls at the ratio of 80% and its histopathology table is like granular cell myoblastoma but pseudoepitheliomatous hyperplasia is not seen in it.Large cells that have eosinophilic cytoplasm forms parenchyma of tumor [6]. CE has a benign prognosis and almost never recurrences [7]. Most of CE are soliter nevertheless there are cases that are large and multiple.…”

Section: Introductionmentioning

confidence: 99%

Congenital Epulis of the Newborn: A Case Report

Pekçetin¹,

Senemtaşı²,

Koçak³

et al. 2018

OJST

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Background: Congenital epulis (CE) also known as congenital granuler cell tumor is a rarely encountered pathology the majority of which originates from the gingival mucosa, particularly the anterior portion of the maxillary alveolar ridge. CE mostly seen in girls. CE with unclear histogenesis and etiology is seen at birth as a solitary mass in oral cavity. Apart from non-congenital epulis, it contains granular cells. So lesion is named congenital granular cell tumor. CE has a benign histopathology and after surgery there is no recurrence reported in the literature. Aim: The purpose of this case report, is to present, 5 day-old female neonatal girl who was seen CE on the left maxillary alveolar ridge on the region of the future incisors. Case Presentation: The tumoral lesion was well-circumscribed and 10 mm in diameter, smooth surfaced and red in colour much like alveolar mucosal tissue. Tumoral lesion was affecting oral feeding due to obstruction. Excisional biopsy was performed under topical anesthesia. The histopathology was reported as congenital epulis. During the 4 months follow-up, we have seen no complication. Conclusion: CE is a neonatal congenital tumor which is very rare. The treatment of CE is surgical excision. Unless the early treatment is not executed, tumor may cause difficulties in oral feeding and respiration. Therefore it should be excised in an early period.

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Congenital epulis of the newborn

Cited by 29 publications

References 17 publications

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion

Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion

Congenital granular cell epulis: A case series

Congenital Epulis of the Newborn: A Case Report

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