*Ewa Krasuska-Sławińska1, Dariusz Polnik2, Dorota Olczak-Kowalczyk3, Tomasz Dzik4, Katarzyna Świstak1
Congenital granular cell tumour – two case reports
Wrodzony guz ziarnistokomórkowy – opis dwóch przypadków
1Dental Surgery Clinic, The Children’s Memorial Health Institute
Head of Clinic: Agnieszka Pieniak, MSc
2Department of Paediatric Surgery and Organ Transplantation, The Children’s Memorial Health Institute
Head of Department: Professor Piotr Kaliciński, MD, PhD
3Department of Paediatric Dentistry, Medical University of Warsaw
Head of Department: Professor Dorota Olczak-Kowalczyk, MD, PhD
4Department of Pathology, The Children’s Memorial Health Institute
Head of Department: Associate Professor Maciej Pronicki, MD, PhD
Wrodzony guz ziarnistokomórkowy jest to rzadko występująca zmiana o łagodnym charakterze, najczęściej pojedyncza, uszypułowana, wyrastająca z przedniej części wyrostka zębodołowego szczęki, rzadziej części zębodołowej żuchwy. Guzy o znacznych rozmiarach mogą utrudniać odżywianie i oddychanie, dlatego wymagają interwencji chirurgicznej, polegającej na ich usunięciu. W przypadku zmian o niewielkich rozmiarach możliwa jest spontaniczna regresja.
Celem pracy jest przedstawienie dwóch pacjentek, u których bezpośrednio po urodzeniu stwierdzono obecność uszypułowanych guzów w jamie ustnej.
Dwa noworodki płci żeńskiej, leczone w Klinice Chirurgii Dziecięcej i Transplantacji Narządów IP-CZD z powodu guzów w obrębie wyrostka zębodołowego szczęki/części zębodołowej żuchwy zdiagnozowanych bezpośrednio po urodzeniu, niewidocznych w badaniach USG wykonywanych w czasie ciąży. Obydwa guzy o średnicy około 1,5 cm łatwo ulegały urazom podczas ssania, powodując krwawienie, dlatego podjęto decyzję o ich chirurgicznym usunięciu. W materiale histopatologicznym uzyskano obraz wrodzonego guza ziarnistokomórkowego noworodków. Badanie kontrolne obu pacjentek po 6 miesiącach wykazało prawidłową budowę wyrostka zębodołowego szczęki/części zębodołowej żuchwy.
W przypadku guzów ziarnistokomórkowych o dużych rozmiarach leczeniem z wyboru jest leczenie chirurgiczne. Guz nie powoduje zmian w budowie wyrostka zębodołowego szczęki/części zębodołowej żuchwy.
Congenital granular cell tumour is a rare, usually single, pedunculated benign lesion, originating from the anterior part of the maxillary alveolar process, less frequently from the alveolar part of the mandible. Large tumours can cause problems in eating and breathing, therefore require surgical intervention involving excision. Smaller lesions can resolve spontaneously.
The aim of the study was to present 2 case reports of patients with pedunculated oral tumours diagnosed immediately after birth.
Two female newborns treated at the Department of Paediatric Surgery and Organ Transplantation (CMHI) due to tumours located within the maxillary alveolar process/alveolar part of the mandible, which were diagnosed immediately after birth, but were not visualised during prenatal ultrasound. Both tumours with a diameter of about 1.5 cm were easily damaged during sucking, causing bleeding. Therefore a decision was made to perform their surgical removal. Histopathological examination revealed congenital granular cell tumour of the newborn. A follow up after 6 months showed normal structure of the maxillary alveolar bone/alveolar part of the mandible.
Surgery is the treatment of choice in patients with large granular cell tumours. The tumour does not cause changes in the structure of the alveolar process/the alveolar part of the mandible.
Congenital granular cell tumour was for the first time described by Neuman in 1871 as a rare benign lesion of unknown aetiology, eight times more common in female compared to male newborns (1). Clinically, it appears as a single pedunculated tumour originating from the anterior part of the maxillary alveolar process, mostly from the gingival papilla, less often from the alveolar part of the mandible. Large tumours can cause problems in eating and breathing, therefore require surgical intervention involving their excision. Smaller lesions can resolve spontaneously. Histologically, congenital granular cell tumour is identical to granular cell tumour, also known as Abrikosoff’s tumour, which typically occurs in adults, mostly on the tongue. Their differentiation is based on immunohistochemistry and electron microscopy. Differential diagnostics should also include neuroectodermal tumors, hemangiomas, teratomas, fibromas, rhabdomyomas, odontogenic tumours, dermoid cysts (1, 2).
A 3-day-old female newborn was admitted to the Department of Paediatric Surgery and Organ Transplantation (CMHI) due to an exophytic lesion present in the anterior part of the maxillary alveolar process. The girl was born from the first pregnancy, first delivery by Caesarean section due to lack of progress in labour at 39 weeks gestation, in overall good condition (Apgar score of 10). Medical history of normal course of pregnancy, maternal group B streptococcal (GBS) genital tract colonisation managed with antibiotic prophylaxis.
Physical examination of the oral cavity revealed an exophytic tumour with a broad pedicle in the anterior part of the maxillary alveolar process (fig. 1). The lesion measuring 1.5 x 1.5 x 2 cm did not prevent sucking or breathing, however, it was easily damaged causing bleeding when sucking. Other than that, normal pink smooth and shiny oral mucosa without pathological lesions. Blood tests (blood count, coagulation parameters, ionogram, blood type), abdominal and CNS ultrasound revealed no abnormalities.
Fig. 1. Case 1. Maxillary alveolar tumour
The entire polypoid tumour of the alveolar soft tissues was removed at 4 hours of life, under general anaesthesia with endotracheal intubation using a bipolar diathermy (fig. 2). The wound was closed with absorbable sutures. The postoperative period was uneventful.
Fig. 2. Case 1. Maxillary alveolar tumour post-excision
Histopathological examination revealed gingival granular cell tumour of the newborn. Immunochemistry was also performed: CD68(+), AE1/AE3(-), CD34(+), S-100(-). The patient has remained under observation at the Surgery Outpatient Clinic for Children and Adults: six months after surgery the child develops normally. Intra- and extraoral clinical examination revealed no abnormalities. Normal vestibular and oral mucosa. Symmetrical maxillary alveolar process with no pathological lesions (fig. 3).
Fig. 3. Case 1. Maxillary alveolar process 6 months after surgery
A one month-old female infant was referred by a paediatrician to the Surgery Outpatient Clinic for Children and Adults (CMHI) for consultation regarding an exophytic lesion in the mucosa of the anterior alveolar part of the mandible, on the left side. The lesion was present from birth and did not impair breathing or eating. However, it was easily damaged during feeding. The girl was born from the second pregnancy, second vaginal delivery at 39 weeks gestation; she received an Apgar score of 10. Unremarkable prenatal and perinatal history; negative maternal GBS.
Clinical examination of the oral cavity revealed a pedunculated tumour with a diameter of about 1.5 cm, covered with unchanged mucosa, present in the anterior alveolar part of the mandible (fig. 4). Due to bleeding during feeding the patient was qualified for surgical removal of the lesion. The child was admitted to the Department of Paediatric Surgery and Organ Transplantation for surgery. Preoperative blood tests (blood count and coagulation parameters, ionogram, blood type) showed no abnormalities.
Fig. 4. Case 2. Tumour of the alveolar mucosa of the mandible
The lesion was resected within healthy tissue margins, under general endotracheal anaesthesia using a bipolar diathermy (fig. 5). The postoperative period was uneventful.
Fig. 5. Case 2. Tumour of the alveolar mucosa of the mandible – status post-excision
Histopathological examination revealed granular cell tumour of the newborn. Immunohistochemistry: CD68(+), S-100(-/+), CD3(-/+). Normal further development of the child (fig. 6). The patient has remained under observation at the Surgery Outpatient Clinic for Children and Adults (CMHI).
Fig. 6. Case 2. The alveolar part of the mandible 1 week after surgery
Neonatal oral tumours are very rare. Most literature reports describe teratomas and congenital epulis. Congenital epulis, also known as congenital granular cell tumour (due to the possible occurrence of a tongue tumour with similar tissue) or Neumann’s tumour, is a rare benign gingival tumour. It is usually found on the edge of the maxillary alveolar process, as in the first described case. These lesions are rarely found in the mandible (three times less frequently compared to the maxilla). In the second described patient, the tumour was located in the anterior alveolar part of the mandible. This location is sporadically described in the literature (3). The aetiology of the tumour is unknown. It is reported in the literature that the incidence of such neonatal lesions is 8 to 10-fold higher in females compared to males, which may indicate the involvement of hormonal factors in the development of these lesions (3, 4). Similarly as in our patients, these are mostly single tumours, rarely occurring as multiple lesions (3, 5). Tumours of various sizes have been described – from a few up to 90 mm. Large lesions can cause respiratory impairment and prevent normal neonatal feeding (6-8).
Although prenatal diagnostics seems to play a major role, this type of tumours are rarely detected in prenatal tests (8, 9). Although prenatal ultrasound allows the assessment of tumour size, evaluation of these lesions in only possible in the third trimester of pregnancy (5). Nevertheless, it may prove crucial for the perinatal management. In extreme cases of very large tumours, it may be necessary to intubate a newborn before cutting the umbilical cord (EXIT method) (2, 10).
Prenatal testing failed to detect the presence of lesions in both our patients. The tumours were diagnosed after birth. In the case of impaired breathing or feeding due to large size of the granular cell tumour as well as bleeding while feeding, surgical removal is necessary. According to literature, total excision of the tumour is not required due to its benign character as well as due to the lack of recurrence or distant metastases (4, 11). There are even reports suggesting that excessively radical excision can lead to deformation of surrounding tissues and the damage of tooth buds (6, 11, 12). Some authors recommend lesion monitoring instead of surgical intervention in smaller lesions due to the possibility of their spontaneous regression (11, 13). In both girls, the tumours were easily damaged, causing bleeding; therefore it was decided to perform their surgical excision. In both cases, bipolar diathermy was used for resection, ensuring a very good hemostasis and a minimal risk of tooth bud damage.
Although distant observations indicate that these tumours generally do not cause dental abnormalities and are not accompanied by other congenital disorders, several cases of hypoplasia or absence of teeth and tissue deformation around a large tumour have been described. The follow-up period in our patients was 6 months, therefore only morphological assessment of the maxillary alveolar process/the alveolar part of the mandible was possible. In both girls, no abnormalities were found in the shape or structure of the maxillary alveolar process/the alveolar part of the mandible. Due to young patient age and normal shape/size of the maxillary alveolar process and the alveolar part of the mandible, there were no indications for radiological diagnostics for the presence or absence of deciduous tooth buds. Both patients remain under constant care of Surgery Outpatient Clinic for Children and Adults (CMHI). Another scheduled follow-up visit will take place in six months.
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