Congenital Oral Masses: An Anatomic Approach to Diagnosis.

Although congenital oral masses are rare, they are readily detectable during fetal US screening. Most congenital oral masses are benign, but some may cause mechanical airway obstruction, resulting in poor outcomes at delivery. The radiologist's ability to describe these abnormalities and their physiologic sequelae accurately can have a substantial effect on perinatal treatment. Furthermore, despite being rare, congenital oral lesions encountered at screening and at follow up fetal MRI provide the opportunity to make a specific diagnosis by following a simple anatomic approach. This article describes an anatomic algorithm as the framework for accurate diagnosis of congenital oral lesions. The imaging appearance of the most common congenital oral cavity neoplasms is outlined, including vascular anomalies, epulides, choristomas, congenital lingual thyroid anomalies, lingual hamartomas, and epignathi, and other conditions that mimic these at US. Also reviewed are perinatal management of masses that affect the fetal airway and the imaging features key to optimizing delivery outcomes. Online supplemental material is available for this article. ©RSNA, 2019.

Congenital Granular Cell Tumor of the Arm: A Rare Presentation.

Congenital granular cell tumors are uncommon benign tumors of newborns that mainly affect oral mucosa, especially the maxillary alveolar ridge. They are predominantly seen in female newborns, and cutaneous involvement is extremely rare. In this report, we present a case of congenital granular cell tumor on the arm of one of the male monozygotic twins and discuss the differential diagnosis of granular cell phenotype.

Congenital granular cell lesion in newborn mandible.

Congenital granular cell lesion (CGCL) is a rare non-neoplastic lesion found in newborns also known as Neumann's tumor. This benign lesion occurs predominantly in females mostly as a single mass. The histogenesis and natural history of the lesion remains obscure. It arises from the mucosa of the gingiva, either from the maxillary or mandibular alveolar ridge. The lesion is more common in the maxillary alveolar ridge than the mandibular.The present report describes a case of congenital granular cell lesion in an eight-day-old female child who was born with a mass on the anterior mandibular alveolar ridge. The mass was protruding from her mouth and compromised feeding. A clinical diagnosis of teratoma was suggested. Histologically, cells of this lesion are identical to granular cell tumor (neuroectodermal type) and show intense diastase-resistant Periodic Acid Schiff positivity. Immunohistochemically, cells are positive for vimentin but negative for S-100 and desmin, thus suggesting that CGCL is possibly derived from primitive gingival mesenchymal cells rather than having schwannian origin.

Congenital epulis of the newborn: 10 new cases of a rare oral tumor.

Congenital epulis of the newborn (CEN) is a rare benign lesion that exclusively occurs in the oral and maxillofacial regions of newborns. The clinicopathologic features of CEN were examined and reviewed from the files of the Armed Forces Institute of Pathology from 1970 to 2000. Ten cases were included. Patient lesions were all present at birth but were surgically excised between 2 days and 6 weeks (median, 5.5 days). Nine lesions were in females; 1 case did not designate patient sex. Locations included 6 on the maxilla, 2 on the mandible, 1 on the designated maxillary lip, and 1 unknown. The cases included a patient with 2 lesions: 1 on mandibular and 1 on maxillary alveolar ridges, respectively. All other lesions were solitary and polypoid. Microscopically, these were pedunculated and nodular, composed of sheets to grouped clusters of medium-sized, ovoid-to-polygonal cells with abundant granular cytoplasm, distinct cell membranes, vascular-rich stroma, and attenuated overlying mucosa. Two cases also demonstrated spindled cells. The nuclei were vesicular and focally stippled, with distinct and slightly convoluted nuclear membranes; nucleoli were visible but not prominent. Mitotic activity was not observed. The vascular channels ranged from capillary-sized to venous, some staghorn-like with rare perivascular long-term inflammation. The venules exhibited a perivascular pericytic proliferation. Odontogenic epithelial rests were present in 2 cases. No cases demonstrated cytoplasmic hyaline globules. The lesional cells in all cases were negative for S-100 protein, CD68, CD34, CD31, keratins, desmin, calponin, and smooth muscle actin. Perivenular pericytes were positive for smooth muscle actin. Congenital epulis of the newborn is a rare oral entity with characteristic clinicopathologic features. It predominately affects girls, mainly on the maxillary alveolar ridge. It may be separated from "granular cell tumor" by location, patient age, absence of cytoplasmic hyaline globules, solid growth pattern, pericytic proliferation, attenuated overlying epithelium, and negativity for S-100 protein.

Multiple congenital epuli (congenital granular cell tumours) in the newborn: a case report and review of literature.

Congenital epulis of newborn is very rare benign intraoral entity of uncertain ethiology. Histologically the lesion is similar to the granular cell tumour of an adult but immunohistochemical stainings prove their different origin. Treatment involves surgical excision, recurrences are rare.

Congenital granular cell lesion: clinical, microscopic and immunohistochemical aspects in a case of multiple lesions.

Congenital granular cell lesion of the newborn, also known as congenital epulis, is a rare benign oral cavity tumor presenting at birth. Usually, it appears as a solitary mass arising in the mouth and originates from the anterior alveolar ridge. The objective of the present article is to report a case of congenital granular cell lesion in an 8-day-old female newborn. The patient presented four intraoral pedunculated lesions. Diagnosis, treatment, microscopic and immunohistochemical characteristics are also discussed.

[Antenatal diagnosis of a congenital granular cell tumor]. / Tumeur granuleuse gingivale congénitale de découverte anténatale.

INTRODUCTION: Neuman's tumors also called congenital epulis or congenital gingival granulomatous tumors are rare and benign oral cavity tumors. They are usually discovered at birth. CASE REPORT: A 32 year-old pregnant female patient was followed for gravid diabetes well controlled. A maxillary tumor in the fetus was revealed by the third trimester US. A fetal MRI was prescribed, after discussion with the antenatal diagnostic center, to determine the localization, size, and nature of the tumor. It revealed a 3 cm long tumor with cystic like areas not communicating with the encephalus. A caesarian section was performed 2 weeks before term. A simple tumoral excision was performed at 12 hours of age. Histological analysis confirmed the diagnosis. There was no local recurrence after 6 months of follow-up. DISCUSSION: A prenatal diagnosis of congenital gingival granulomatous tumor is rare. It is usually made at the third trimester ultrasound scan, rarely with fetal MRI. Nevertheless, prenatal diagnosis allows for a better postnatal management.

Congenital epulis in the newborn, review of the literature and a case report.

Congenital epulis (CE) is a rare tumor of the newborn, also known as granular cell tumor or congenital gingival granular cell tumor because of its histologic features. Neumann first described CE in 1871. Epulis is seen only in the newborn and is a different entity from other granular cell tumors. CE has a female predilection with 8:1 ratio. Epulis is most frequently located on the anterior maxillary alveolar ridge and usually occurs as a single mass although 10% cases occur as multiple. CE clinically appears as a pedunculated protuberant mass, which may interfere with respiration or feeding. In cases with large lesions mechanical oral and nasal obstruction can impair fetal deglutition and neonatal respiratory efforts resulting in polyhydramnios prenatally or respiratory impairment postnatally. The exact histiogenesis is still uncertain. The recommended treatment is prompt surgical resection. Recurrences of the tumor and damage to future dentition have not been reported. Spontaneous regression of congenital epulis has been reported in a few cases. However, surgical excision is generally indicated due to interference with feeding or respiration. We report a female neonate with congenital multiple tumors in the anterior maxillary alveolar ridge and review the relevant literature. The tumors were resected by surgical excision. The intraoperative and postoperative course was uneventful. Follow-up for 6 months did not show recurrence.

Congenital granular cell tumor.

Congenital granular cell tumor or congenital epulis is a rare benign soft tissue lesion in newborns. It usually arises from the mucosa of the gingiva, either from the maxillary or mandibular alveolar ridge, and may cause respiratory or feeding problems. We reported a case of a 1-hour-old female newborn with a congenital granular cell tumor in the anterior maxillary alveolar ridge. The lesion was causing a feeding problem and was excised under general anesthesia when the newborn was 1 day old.

[Congenital granular cell epulis: a case report]. / Kongenitální epulis z granulárních bunek: popis prípadu.

Congenital granular cell epulis is a rare benign lesion occuring exclusively in newborns as a solitary or multiple swelling of alveolar mucosa of either maxilla or mandible. A case of a female newborn who presented with multiple swellings of both maxillary and mandibular alveolar mucosa measuring up to 10 mm is presented. On 24th postnatal day, the lesions were under general anesthesia excised and sent for microscopic examination, which revealed congenital granular cell epulis. Immunohistochemically, granular cells showed expression of both vimentin and PGP 9.5, whereas the proof of S100 protein and inhibin was negative. The postoperative course was uneventful and follow-up for 8 months showed no local recurrence.

Congenital epulis. A case report.

Congenital epulis (CE) is an uncommon granular cell lesion of unknown pathogenesis and uncertain histogenesis that presents at birth. It is a benign hamartoma that may interfere with feeding and respiration. The treatment of CE is by simple excision. In the 136 years since CE was first described, more than 200 cases of CE have been reported in the literature.

Congenital granular cell tumor of the newborn - Spontaneous regression or early surgical intervention.

Congenital granular cell tumor of the newborn is a benign, relatively innocuous hamartomatous lesion arising from the alveolar ridge. Large lesions often warrant prompt surgical removal owing to the associated difficulty in breastfeeding and respiration of the infant. However, for medium-sized lesions, another approach could be observed for spontaneous regression. The present case report highlights the use of a combination approach for management of tumor in a 2-day-old female child presenting with a large protruding mass from the mouth associated with difficulty in breastfeeding. A combination of observation for regression in size followed by electrosurgical removal of the tumor was done. The infant was followed up regularly over a long period of 3 years to assess any adverse effects of surgical intervention on the maxillary primary anterior dentition of which none were recorded.

Congenital granular cell tumor (congenital epulis): a lesion of multidisciplinary interest.

Congenital granular cell tumor (CGCT), or congenital epulis, is a very uncommon benign soft tissue lesion that usually arises from the alveolar mucosa of neonates and may cause respiratory and feeding problems. We report a case of a 3-day-old female newborn, who presented an intraoral tumor mass which was protruding from her mouth, and compromising feeding. Under general anesthesia, the lesion was completely removed and the patient had an uneventful postoperative course. Clinical features and treatment approaches are presented and discussed, emphasizing the necessity of a multidisciplinary approach in such cases.

Congenital epulis: a surprise in the neonate.

A newborn infant with congenital epulis can be a striking sight for both parents and health care professionals involved in neonatal care. These tumours of the infant mouth can be remarkably large, occupying much of the oral cavity and posing a risk of airway obstruction or interfering with feeding. Dentists should be able to recognize these swellings as they may be asked to consult and provide information to parents and other practitioners regarding treatment of these lesions.

Congenital epulis - congenital granular cell lesion: a case report.

Neumann first described congenital epulis (CE) of newborn in 1871. CE occurs at birth and has a striking predilection for females. It is most frequently located on the anterior maxillary alveolar ridge. CE clinically appears as a pedunculated protuberant mass. In cases with large lesions mechanical oral and nasal obstruction can impair fetal deglutition and neonatal respiratory efforts resulting in polyhydramnios prenatally or respiratory impairment postnatally. Histologically CE shows characteristic large cells with granular cytoplasm and spindle cells resembling fibroblasts. The exact histiogenesis is still uncertain, various theories of origin are epithelial, undifferentiated mesenchymal cells, pericytes, fibroblast, smooth muscle cells and nerve related cells. A newborn female child with CE in the maxillary alveolar ridge reported to our institution is presented with investigation, management and follow-up.

Congenital granular cell lesion of the tongue: a report of two cases and review of the literature.

The congenital granular cell lesion most commonly occurs on the maxillary or mandibular alveolus of neonates. Extra-alveolar congenital granular cell lesion is exceptionally rare, with only 10 cases reported. Two additional cases occurring on the tongue are presented with a description of the clinical, histopathologic, and immunohistochemical features. The differential diagnosis is discussed, and the literature reviewed.

A study of cell proliferation using immunohistological staining: A case report of congenital granular cell epulis.

An unusual case of a 1-day-old Chinese female neonate with a solid tumor mass in the maxillary anterior ridge of the edentulous jaw is reported. Based on the clinical and histopathological features, the diagnosis was of obstructive congenital granular cell epulis (CGCE) which is an uncommon benign tumor that preferentially develops in female infants. Immunohistochemical analysis of the lesion was performed and the rate of cell proliferation was determined by immunostaining with Ki-67 and PCNA, which showed labeling indexes of 11.1% and 33.3%, respectively. No recurrence was observed in the follow-up.

Congenital granular cell tumor with systemic involvement. Immunohistochemical and ultrastructural study.

We present an autopsy case of a 29-week-old male fetus with a very unusual set of congenital granular cell tumors, including gingival epulis and granular cell lesions of the kidneys, lung, heart, esophagus, small and large intestine, thyroid, adrenals, spleen, urinary bladder, testis, pituitary, and leptomeninges. The granular cells were distributed mainly through the stroma of the organs, but they also involved the epithelial lining of the seminiferous and renal tubules. Ultrastructurally, the gingival, pulmonary, and renal tumors were basically the same in appearance as membrane-bound heterogeneous bodies. Immunohistochemical studies were negative for S100 protein, lysozyme, alpha 1-antitrypsin, cytokeratin, and vimentin in the gingival mass as well as in other systemic lesions. The immunohistochemical reaction pattern of the granular cells in our case was more like the cells of the congenital granular cell epulis rather than adult granular cell tumor because of its negative reaction to S100 protein. However, the involvement pattern was that of the adult form of granular cell tumor. Several developmentally different cells, such as renal tubular epithelial cells, seminiferous tubular cells, gingival stromal cells, and parenchymal cells of many organs, were involved in this granular cell process. The myofibroblastlike cells seen in the region of segmental dysplasia of the kidney showed the same cytoplasmic material as in typical granular cells. Based on these findings, it is suggested that a histogenesis of multiple cell origin of the granular cell tumor could be strongly supported.

A rare case of multiple congenital epulis.

We report a case in a female newborn infant of multiple congenital epulis, i.e. granular cell tumor, that was undetected during regular pregnancy ultrasound monitoring. At birth the neoplasms appeared as two voluminous lesions protruding from the newborn's mouth. The greater of them (5.5 cm x4 cm x3 cm) was pedunculated and attached to the external superior gingiva, shifting the alae nasi and making it difficult to enter the coanae. The second mass was somewhat smaller (3 cm x4 cm x2.5 cm), pedunculated and attached to the external inferior gum. A third smaller mass was less evident, unpedunculated and attached to the rim of the lower gingiva. Histologically the lesions were characterized by large cells, which had abundant pale acidophilic granular cytoplasm. A round-oval nucleus was located centrally. The cell membranes were distinct. Neither mitosis nor necrosis was found. Staining for cytoplasmic granules was intensely periodic acid-Schiff (PAS) positive and diastase resistant. Immunohistochemical negativity for S100 protein, positivity for lysozyme and numerous phagolysosomes in the cytoplasm of neoplastic elements, observed on ultrastructural examination, supported the hypothesis that the congenital type of granular cell tumor cannot have a Schwannian origin like that of the adult type, but is probably a mesenchymal lesion which, for unknown cause, regresses by a degenerative process.

Obstructive congenital gingival granular cell tumor.

Congenital gingival granular cell tumors (also known as congenital epulis or Neumann's tumor) are rare and always benign intraoral tumors originating from the alveolar ridge. They are typically seen as a mass protruding out of a newborn child's mouth. We report a case of a large obstructive congenital gingival granular cell tumor of the mandibular ridge. The intraoral mass was first detected on a 38-week prenatal ultrasound scan and resulted in neonatal airway obstruction. Complete surgical removal was performed with an uneventful postoperative course. Histologic and immunohistochemical assessments with antibodies against S-100 protein confirmed the diagnosis. The clinical and morphological aspects, differential diagnosis, histogenesis, and treatment are discussed.