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Abstract:Ganglioneuroma is a benign tumor that originates from primordial neural crest cells which migrate from the mantle layer of the developing spinal cord to the sympathetic ganglia, adrenal medulla, and other sites. The most affected anatomical sites are the posterior mediastinum, retroperitoneum, adrenal gland, head and neck. It occurs most commonly in children over 10 years of age and consists of ganglion and Schwann cells. We report a case of 9 – year- old male child who presented with mass per abdomen in the right hypochondrium. Fine needle aspiration cytology showed mature ganglion cells and cluster of Schwann cells suggestive of Ganglioneuroma. Cytology diagnosis was confirmed by histopathological examination of tru-cut biopsy and subsequently excised specimen. On regular follow he is doing fine without any symptoms.
Abstract:Ganglioneuroma is a benign tumor that originates from primordial neural crest cells which migrate from the mantle layer of the developing spinal cord to the sympathetic ganglia, adrenal medulla, and other sites. The most affected anatomical sites are the posterior mediastinum, retroperitoneum, adrenal gland, head and neck. It occurs most commonly in children over 10 years of age and consists of ganglion and Schwann cells. We report a case of 9 – year- old male child who presented with mass per abdomen in the right hypochondrium. Fine needle aspiration cytology showed mature ganglion cells and cluster of Schwann cells suggestive of Ganglioneuroma. Cytology diagnosis was confirmed by histopathological examination of tru-cut biopsy and subsequently excised specimen. On regular follow he is doing fine without any symptoms.
Ganglioneuroma (GN) is a rare benign neurogenic tumor which arises from the neural crest cells that represent the final maturation stage of neuroblasts. GN is commonly encountered in adolescent or young adult. Until now, only a few cases of intrathoracic GN were reported in pediatric patients, especially in below 10 years of age. We report here an 8-year-old boy, who first presented to primary care with symptoms of upper respiratory tract infection. Radiologic investigations revealed a giant posterior mediastinal mass extending from T4 to T9 vertebrae without evidence of vascular invasion or infiltration into adjacent structures. The patient subsequently underwent left thoracotomy and resection of mass under general anesthesia. He was discharged 3 days after operation without complication. Histopathologic examination confirmed the diagnosis of thoracic GN. Grades of the neuroblastic differentiation increase with the median age at diagnosis. Although GNs are usually benign, they can grow aggressively and cause compression to an adjacent structure. Therefore, surgical resection is the only treatment. Debulking of tumor provides an alternative solution, especially when vital structures are involved. Complete resection remains the gold standard treatment for GN. However, in cases of incomplete resection, all residual tumors require regular clinical and radiodiagnostic follow-up.
Rationale: Ganglioneuromas are benign neoplasm of neuroblastic origin which arise from central or peripheral parts of the autonomic nervous system. They are normally found at posterior mediastinum, retroperitoneum, and the adrenal gland but ganglioneuromas are rarely found in the cervical region. Patient concerns: A 12-year-old boy was admitted with a left-lateral neck mass slow growing over a 7-days duration. The tumor was painless and was not associated with any systemic or compression-related symptoms. No symptoms of Horner's syndrome, including ptosis, myosis, ipsilateral facial anhidrosis, and flushing, were observed. Laboratory routine tests were within normal limits, and magnetic resonance imaging demonstrated a solid and well-circumscribed mass in the carotid space. Diagnosis: Due to the patient's symptoms, laboratory test results together with radiographic investigation findings, the 12 years old boy was diagnosed with cervical ganglioneuroma combined with tetralogy of Fallot. Interventions: Surgical excision. Outcomes: The postoperative period was uneventful with the exception of Horner's syndrome on the left side in short period, and it was finally resolved after 8 months recovery. The patient is now in stable condition after operation, with improvement in symptoms during follow-up recovery. Lessons: Ganglioneuromas should be accounted as the differential diagnosis of pediatric soft tissue tumors of the head and neck. The diagnosis for ganglioneuromas in cervical region can only be ascertained with postoperative pathologic examination, and excision is considered as the only effective treatment modality known so far which may cause Horner's syndrome at times. However, patients have a favorable prognosis without recurrence overall.
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