|Year : 2013 | Volume
| Issue : 1 | Page : 96-98
Adrenal neuroblastoma in an adult
Phalguni Gupta1, Abhishek Maiti2, Ranen K Aich3, Asit R Deb3
1 Department of Radiotherapy, Midnapore Medical College; Department of Medicine, Nilratan Sircar Medical College, Kolkata, India
2 Department of Medicine, Nilratan Sircar Medical College, Kolkata, India
3 Department of Radiotherapy, Nilratan Sircar Medical College, Kolkata, India
|Date of Web Publication||10-Apr-2013|
Nilratan Sircar Medical College, Kolkata-700014, West Bengal
Source of Support: None, Conflict of Interest: None
Adrenal neuroblastomas, although quite common in children, are extremely rare in adulthood. Here, we are reporting the case of a 47-year-old male who presented with right flank pain and had a palpable mass in the same region. Contrast-enhanced computed tomography showed an irregular, poorly marginated heterogeneous mass lesion arising from the right suprarenal position. Urinary catecholamines were within normal limits. There was no evidence of metastatic disease. The mass was resected en-block along with the right kidney. Histopathologic examination revealed the diagnosis of a neuroblastoma. Adjuvant chemotherapy was instituted, but the pain recurred after 9months. Despite subsequent chemotherapy, the mass continued to grow over the next 6months with further infiltration into the liver and surrounding muscles. The patient has currently been started on external palliative radiation. We have also reviewed the literature to present a discussion on presentation, diagnosis, and management of this rare tumor.
Keywords: Adult, neuroblstoma, adrenal gland
|How to cite this article:|
Gupta P, Maiti A, Aich RK, Deb AR. Adrenal neuroblastoma in an adult
. J Can Res Ther 2013;9:96-8
| > Introduction|| |
Neuroblastomas are malignant tumors derived from primitive neural crest cells. It is the most common extracranial malignant tumor of childhood, with approximately 90% of patients presenting before 5years of age. , However, neuroblastomas are extremely rare in adults (≥20 years), with no more than 65 cases reported till 2008.  An update of the search revealed only a handful of additional cases. We herein present a case of a 47-year-old malen who was diagnosed with neuroblastoma and is currently undergoing palliative radiation therapy.
| > Case Report|| |
A previously healthy 47-year-old Indian male presented with right flank pain of 3 months duration. Physical examination suggested the presence of a right upper quadrant mass. The routine laboratory tests (complete blood count, liver, and kidney function tests) were within normal limits; but urinalysis revealed microscopic hematuria. Cystoscopy was unremarkable. Abdominal ultrasonography revealed a solitary retroperitoneal mass arising from the upper pole of the right kidney and excretory urography showed a mass effect superiorly on the right kidney. Contrast-enhanced computed tomography (CECT) showed an irregular, poorly marginated heterogeneous mass lesion arising from the right suprarenal position, with invasion into the upper pole and extending up to the hilum of the right kidney [Figure 1] and [Figure 2]. Further laboratory investigations revealed normal levels of dehydroepiandrosterone (DHEAS), 17-hydroxyprogesterone, testosterone, free testosterone, aldosterone, cortisol, plasma renin activity, 24-h urinary levels of vanillyl mandelic acid, and metanephrine. Serum LDH level was raised (291 U/L). Chest radiographs and bone scans showed no evidence of metastatic disease.
|Figure 1: Axial contrast-enhanced computed tomography showing a right suprarenal mass invading the upper pole of the right kidney|
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|Figure 2: Axial contrast-enhanced computed tomography showing the right suprarenal mass extending up to the hilum of the right kidney|
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Subsequently, with the provisional diagnosis of a suprarenal malignancy or a renal cell carcinoma, the patient underwent surgery with prophylactic α-adrenergic blockade. The large suprarenal mass was removed along with the right kidney due to gross invasion. Histopathologic examination revealed sheets of small, dark cells having high nuclear-to-cytoplasm ratio divided by fibrous septa and scanty Schwannian stroma [Figure 3] and [Figure 4]. Thus the mass was found to be a stroma poor group of neuroblastoma, probably unfavorable subtype (as per histopathologic prognostic factors),  arising from the right adrenal gland. Six dissected lymph nodes showed no signs of metastasis. As resection margins as well as representative ipsilateral lymph nodes were microscopically negative for disease, as per International Neuroblastoma Staging System (INSS), the disease was of Stage-1. MYCN status, Ki-67 leveling index, and NSE level estimation were suggested but could not be done due to lack of infrastructure.
|Figure 3: Section at higher magnification showing sheets of small, dark cells having high nuclear-to-cytoplasm ratio (H and E, × 400)|
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|Figure 4: Section showing a tumor mass composed of sheets of cells divided by fibrous septa and scanty Schwannian stroma (H and E, ×100)|
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As the patient was of stage-1 disease but of undetermined prognosis due to the absence of MYCN status, he underwent 6 cycles of chemotherapy with inj. cisplatin 90mg/M 2 on day 1 as 8 h infusion and inj. etoposide 150mg/M 2 /day as continuous infusion for 3 days; to be repeated from day 22. Radiotherapy was withheld as it was a Stage-1 disease and to avoid radiotherapy-induced toxicities in future.
Although the pain subsided initially, the patient returned 9months later with recurrence of a similar pain. A positron emission tomography-computed tomography (PET-CT) scan this time revealed a mass lesion in the right adrenal area compressing and displacing the head of the pancreas and inferior vena cava [Figure 5]. The mass had also infiltrated into and involved the I, VI, and VII segments of the liver [Figure 6]. There was also peripancreatic and retropancreatic lymphadenopathy. Subsequently, the patient underwent 6 cycles of chemotherapy with irinotecan and carboplatin. Over the next 6months, as revealed by CECT, the mass continued to grow with further infiltration into the liver and surrounding muscles. The patient has currently been started on external palliative radiation (30Gy to be given in 10 fractions over 2 weeks).
|Figure 5: Positron emission tomography-computed tomography showing a mass lesion in the right adrenal area compressing and displacing the head of the pancreas and inferior vena cava and also infiltrating into the liver|
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|Figure 6: Positron emission tomography-computed tomography showing the mass infiltrating into the liver|
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| > Discussion|| |
The overall incidence of adult neuroblastoma in the USA through 1998-2002 was 0.12 cases per million per year with most cases occurring in the third decade of life. , Consequently clinical data regarding presentation, management, and outcome of adult patients with neuroblastoma are scarce and most patients appear to be treated along the pediatric guidelines available. ,,
Adult neuroblastomas often have peculiar features and behave differently than those in infants and children. These generally present at an advanced stage, may have an aggressive to indolent course and worse prognosis; with ultimate survival being less than 5%. , Neuroblastomas commonly arise from the adrenal medulla but may arise from sympathetic ganglia at any site. Common sites of origin in adults include the abdomen, pelvis, mediastinum, thoracic spine, and the olfactory region. , Primary tumors in the neck or upper chest can present as Horner's syndrome. Tumors along the spinal column can cause cord compression, with resulting paralysis. Neuroblastomas typically metastasize through the hematopoietic system. Usual sites of metastasis include the bone, bone marrow, lungs, pleura, brain, breast, liver, and lymph nodes. , Patients generally present with a tumor mass, commonly as in this case, an abdominal mass or pelvic mass or with bone pain due to bone metastasis. Obviously as in this case, for masses arising from the adrenal gland region in adults, other common adrenal tumors or even renal cell carcinoma are more practical differential diagnoses than a neuroblastoma. Unusual modes of presentation include neuroblastoma masquerading as acute leukemia.  Other peculiar biologic features include a decreased incidence of MYCN oncogene amplification, which is a marker for poor outcome in older children. , Only 40%-57% adult patients demonstrate urinary catecholamine elevation compared with 95% of children with neuroblastoma. ,
CECT is the imaging modality of choice as it provides information about the tumor density, local invasion, and distant metastasis and also helps to plan the surgical management. Magnetic resonance imaging is useful for assessing tumors with spinal origin. Radiolabeled meta-iodobenzylguanidine scan and technetium 99m (99mTc) methylene diphosphonate bone scan are extremely useful as these have a high degree of sensitivity and specificity for detecting bone and bone marrow disease.  Ultimately, confirmation of diagnosis necessitates a biopsy from the primary tumor.
Despite the lack of a clear consensus and poor prognosis for adult patients, an appropriate combination of surgery, high-dose chemotherapy and radiation therapy offers the best hope. Surgical intervention should focus on complete resection of the tumor wherever feasible, avoiding any spillage of the tumor. High dose combination chemotherapy has been found to be effective in patients with advanced primary or metastatic disease. Useful chemotherapeutic agents include cyclophosphamide, iphosphamide, vincristine, adriamycin, cisplatin, carboplatin, and etoposide. Bone marrow ablative therapy with bone marrow transplant has been found to be useful in high-risk neuroblastomas.  Radiation therapy is particularly useful in controlling local relapse rate for high-risk neuroblastomas. Due consideration should be given to the stage, molecular markers of the tumor, and existing pediatric guidelines.There is no established role of adjuvant chemotherapy or radiotherapy in stage-1 neuroblastoma in pediatric patients, but the absence of radiotherapy probably led to the early recurrence in this patient and hence should be considered in other cases.
| > Conclusion|| |
Although common in childhood, neuroblastomas are extremely rare in adults. These tumors often have peculiar features and behave differently than those in infants and children. These generally present at an advanced stage, may have an aggressive course and worse prognosis; with ultimate survival being less than 5%. Adjuvant chemotherapy and local radiotherapy should be considered even in early stage disease in adults due to their aggressive nature.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]