|Year : 2015 | Volume
| Issue : 3 | Page : 645
Adrenal neuroblastoma with metastatic mandibular mass: An unusual presentation
Deepak Mittal, Ankur Mandelia, Minu Bajpai, Sandeep Agarwala
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||9-Oct-2015|
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
Neuroblastoma very rarely presents as a mandibular mass. We report the case of a 3-year-old female child who presented to us with a right mandibular mass of 3 months duration. She was investigated and diagnosed as a case of stage 4 right adrenal neuroblastoma with mandibular and skull metastasis.
Keywords: Mandible, metastasis, neuroblastoma
|How to cite this article:|
Mittal D, Mandelia A, Bajpai M, Agarwala S. Adrenal neuroblastoma with metastatic mandibular mass: An unusual presentation. J Can Res Ther 2015;11:645
| > Introduction|| |
Neuroblastoma is the most common extra cranial malignancy of childhood and accounts for 8-10% of all childhood cancers.  It can arise from any site along the sympathetic chain. The route of metastasis is both lymphatic and hematogenous. Hematogenous spread occurs most commonly to bone marrow, cortical bone, liver and skin. Most commonly involved bones are the long bones and the skull bones. It is very rare for neuroblastoma to metastasise in the maxillofacial region. There are few case reports of mandibular metastasis of neuroblastoma but initial presentation with a large mandibular mass is extremely rare. ,,,,,
| > Case report|| |
A 3-year-old female child presented to us with chief complaints of a mass in the right lower jaw region of 3 months duration. The mass was progressively increasing in size. Patient also reported loosening of teeth, fever, anorexia and weight loss. On clinical examination there was a 5 × 5 cm hard, fixed and non-tender mass over the right mandibular region, extending from the angle to the body of the mandible [Figure 1]a. On intra-oral examination there was a 3 × 2 cm mass on the right side with intact buccal mucosa and deranged dentition, which was confirmed on X-ray [Figure 1]b. There was no lymphadenopathy. On palpation of the abdomen, a 12 × 8 cm mass was palpable in the right hypochondrium which was hard, fixed, lobulated and was crossing the midline.
|Figure 1: (a) 5 × 5 cm hard mass over right mandibular region extending from the angle to the body of mandible. (b) X-ray skull showing soft tissue shadow and deranged dentition (black arrow) in the region of the mass|
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Contrast enhanced computed tomography (CECT) of the mandible showed a 5 × 5 cm right mandibular mass with destruction of the underlying bone. CECT of the abdomen revealed a well-defined heterogeneous mass lesion in the right suprarenal region, 10 × 10 cm in size, crossing the midline and the right adrenal could not be seen separately [Figure 2]a. CECT of the chest was normal. Fine needle aspiration biopsy from both masses revealed a small, round blue-cell tumor. The cells were small to intermediate sized having vesicular nucleus with single nucleolus and scanty to moderate amount of acidophilic cytoplasm. Atypical mitosis of 2-3/10 HPF was noted. No neuro fibrillary background was seen. The stroma showed extensive areas of haemorrhage with mild lymphocytic infiltration. Immunohistochmistry revealed positivity for cytokeratin and synaptophysin. Technetium (99mTc)-methylene diphosphonate bone scan showed metastasis to the right half of mandible and to the left parietal bone [Figure 2]b. Bone marrow biopsy and aspiration showed metastatic cells similar to those seen on fine needle aspiration cytology. MIBG (iodine-131-meta-iodobenzylguanidine) scan showed uptake in all three regions with bone marrow involvement. Urinary vanillylmandelic acid and homovanillic acid were elevated at 145 mg/g creatinine and 210 mg/g creatinine, respectively.
|Figure 2: (a) Contrast enhanced computed tomography of the abdomen showing a well-defined heterogeneous mass lesion (black arrows) in the right suprarenal region, 10 × 10 cm in size and crossing the midline, right adrenal could not be seen separately. (b) Technetium (99mTc)-methylene diphosphonate bone scan showing metastasis to the right half of mandible and to the left parietal bone (red arrow)|
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Based on the clinical findings and investigations, the child was diagnosed as a case of stage 4 adrenal neuroblastoma with bony metastasis to the mandible and skull. The patient was categorized as high risk and received intensive induction chemotherapy comprising of cyclophosphamide, doxorubicin, cisplatin and etoposide. She had a partial response to chemotherapy initially and there was significant reduction in the size of both masses. However, the patient expired after 6 months of therapy at the age of 3.5 years due to progressive disease.
| > Discussion|| |
Neuroblastoma is one of the most common solid tumors in infancy and childhood. This neoplasm of neural crest origin, may arise in the adrenal medulla and along the sympathetic ganglion chain from the neck to the pelvis.  In 75% of cases, the tumor is located in the retroperitoneum, in either the adrenal medulla (50%) or the paraspinal ganglia (25%). Less than 5% of tumors occur in the neck or pelvis. 50-75% of reported cases present with an abdominal mass. Presentation could also be related to metastasis to bone, liver, orbit or other sites. 
Based on several clinical series, 50-60% of patients with NB present with disseminated disease (stage IV), a finding that is more likely in patients over 1 year of age.  Metastases to bone marrow and bones have been described in disseminated forms of NB.  Two patterns of marrow disease can be observed: Diffuse type and nodular type.  Bone marrow metastases occur initially in the bone marrow sinusoids where tumor cells adhere and extravasate to bone marrow parenchyma, resulting in the formation of nodular lesions in the medullary cavity. The nodular lesions eventually progress to diffuse lesions after proliferation and gradually enlarge to erode cancellous bone and invade the bone cortex, resulting in bone metastases. 
Considering the age of our patient, the rapid onset of mandibular enlargement together with radiographic features of bone destruction, differential diagnoses included lymphoma, soft tissue sarcoma, osteosarcoma, primitive neuroectodermal tumor, a central malignancy of odontogenic origin, or a metastatic tumor. The presence of an abdominal mass in this patient favored the diagnosis of a metastatic neuroblastoma. However, even after confirmation of the diagnosis of neuroblastoma, it is difficult to comment whether the mandibular lesion was metastatic or a synchronous primary lesion. The bone marrow involvement in our case at the time of the initial presentation and multiple osteolytic lesions in the skull argue strongly for a metastatic origin of the mandibular tumor.
| > Conclusion|| |
An initial presentation of neuroblastoma as a metastatic mandibular mass is rare, but should be considered in a child with a destructive mandibular lesion and constitutional symptoms. Metastatic work up by radiography, bone marrow biopsy and laboratory investigations (e.g. catecholamines) assist with staging and guide treatment in cases of metastatic neuroblastoma.
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[Figure 1], [Figure 2]