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Herein, we detail a multidisciplinary approach and sequential treatment for two infants with congenital granular cell epulis (CGCE). Ultrasonic examinations at 34 weeks of gestation revealed prominent oral masses in both fetuses. To devise a carefully considered treatment strategy, a comprehensive multidisciplinary consultation including oral and maxillofacial surgeons, pediatricians, obstetricians, and anesthesiologists was convened. Following cesarean sections, the lesions were successfully removed, measuring approximately 30 × 15 mm and 30 × 20 mm in size, respectively. Immunohistochemical analysis showed that vimentin was positive, S-100 protein was negative, and NSE protein and CD68 protein were negative. These findings underscore the importance of prenatal diagnosis of congenital granular cell epulis for the effective management of these rare benign conditions.

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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.

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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.

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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.

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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.

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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.

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INTRODUCTION: Congenital granular cell epulis is an uncommon tumor which is apparent at birth. CASE PRESENTATION: Here we report an unusual case of congenital granular cell epulis present in the mouth of a 4-day-old Asian Chinese female newborn. She had six round, soft, multiple, pedunculated swelling masses, of which two were on her upper anterior ridge and four on her lower anterior ridge. The size of the largest lesion was 3.5×3cm, which was causing difficulty in feeding. CONCLUSIONS: The case of a patient with congenital granular cell epulis was reported here because of its rarity. The lesions were surgically removed and satisfactory results were achieved.

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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.

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Congenital granular cell epulis (CGCE) is a very rare benign soft tissue lesion of the neonate, it most frequently located on the anterior maxillary alveolar ridge. It has a female predilection. It is a tumor with no tendency to recur after excision. The exact histogenesis of this tumor remains unresolved and it may be hamartomata.

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A case of an epulis in an 8-month-old boy is reported. The tumor was localized on the mandibular alveolar ridge and, according to the parents, was a recurrence of a congenital tumor excised when the boy was 2 months old. Microscopically, it was composed of many spindled or ovoid cells with vesicular nuclei and non-granular eosinophilic cytoplasm, and covered by acanthotic parakeratinized squamous epithelium with broad rete pegs. Immunohistochemically, the spindled and ovoid cells were intensely positive for vimentin and neuron-specific enolase, and negative for S-100 protein. The final diagnosis was spindle cell epulis. The possible correlation of this lesion with congenital granular cell epulis is discussed.

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Epulis is a rare tumour, with female preponderance that is only seen in the newborns. It arises from the mucosa of the gingiva and protrudes out of the infant's mouth. It can potentially obstruct the airways and may require an EXIT (ex-utero intrapartum treatment) procedure which involves establishing an airway before the feto-maternal circulation is interrupted. We present a female newborn with such a mass, which was diagnosed antenatally. A multidisciplinary team including the neonatologist, anaesthesiologist and ENT specialist should be present in the delivery room to establish the airways, which may require an EXIT procedure. Recommended treatment is early surgical resection. Recurrences of the tumour and damage to future dentition have not been reported, suggesting that radical excision is not warranted.

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The etiology and histogenesis of granular cell tumor are still debated. Granular cell tumor of the newborn is considered to be a different entity than the adult form of this lesion with different immunohistochemical features. We present a case of a rare gingival granular cell tumor in a newborn and review the literature. Gingival granular cell tumor must be clinically differentiated from teratoma, congenital dermoid cyst, congenital fibrosarcoma, hemangioma, lymphangioma, leiomyoma, rhabdomyoma, heterotopic gastrointestinal cyst, congenital cystic choristoma and congenital lipoma. Surface ulceration or pseudoepitheliomatous hyperplasia may lead to confusion with malignancy.

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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.

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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.

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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.

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INTRODUCTION: Congenital epulis, known as a congenital gingival granular cell tumor, is a benign tumor and very rare in newborns. Voluminous or multiple tumors can cause mechanical obstruction of the oral cavity and may result in postnatal feeding and respiratory problems. DISCUSSION: We report the clinical case of a female full-term newborn who presented a tumor on the upper gum obtruding into the oral cavity discovered at birth. The pregnancy was followed normally with three prenatal ultrasounds, which did not show abnormalities. The mass was excised under local anesthesia on the second day of life. The outcome was good after surgery and regular feedings were started on the second postoperative day. Histological examination confirmed the diagnosis of gingival tumor with granular cells and absence of signs of malignancy. CONCLUSION: Prenatal diagnosis is fundamental in the therapeutic approach to this rare lesion but remains difficult because the findings are non specific and the generally late development of the tumor.

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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.

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