|Year : 2014 | Volume
| Issue : 1 | Page : 43-46
Adult onset neuroblastoma, presenting as pleural effusion: A rare entity
Anjan Das1, Abhijit Datta2
1 Department of Pulmonary Medicine, Agartala Government Medical College, Kunjaban, Agartala, West Tripura, Tripura, India
2 Department of Pathology, Agartala Government Medical College, Kunjaban, Agartala, West Tripura, Tripura, India
|Date of Web Publication||5-Feb-2014|
Department of Pulmonary Medicine, Agartala Government Medical College, Kunjaban, Agartala, West Tripura - 799 006, Tripura
Source of Support: None, Conflict of Interest: None
Neuroblastoma of the adrenal gland is an extremely rare tumor in adults. We present a case of 24-year-old male patient, who presented with left sided massive pleural effusion and mass at left upper abdomen. Imaging reported that a huge heterogeneously enhancing mass originating from left adrenal area extended to the left lower chest causing compression of the left lower lobe of lung and pleural effusion. The rarity of this tumor in adults prompted us to present this case.
Keywords: Adult onset, neuroblastoma, pleural effusion
|How to cite this article:|
Das A, Datta A. Adult onset neuroblastoma, presenting as pleural effusion: A rare entity. J Assoc Chest Physicians 2014;2:43-6
| Introduction|| |
Neuroblastoma is the most common tumor in infants, the second most common solid tumor in children.  It is an embryonal malignancy of the sympathetic nervous system arising from neuroblasts, either of sympathetic ganglia of paraspinal region or adrenal medulla. Tumors can develop in the abdominal cavity (about 40% adrenal gland and 25% paraspinal ganglia) or other sites (15% thoracic, 5% pelvic, 3% cervical and 12% miscellaneous).  Adrenal neuroblastoma occurs almost entirely in infants and in children; according to surveillance, epidemiology and end results, the incidence is approximately 9.5 cases/millions of children. Tumors are considered as medical rarity. Virchow is generally credited as being the first to recognize the nature of these adrenal tumors in children.  Neuroblastoma of the adrenal gland is an extremely rare tumor in adulthood. Males have a slightly higher incidence of neuroblastoma than female. ,,, A large number of neuroblastomas undergo spontaneous regression and this could be the reason for scarcity of its presence in the adult population.  Clinical presentation of the disease varies and usually depends on the size of the tumor and involvement of adjacent organs or metastatic sites but may occur without any specific symptoms. More than 90% of patients have elevated catecholamine metabolites (homovanillic acid and/or vanillyl mandelic acid) level detectable in urine, but hypertension is rare.  Histologically, the tumor is composed of small round cells with scanty cytoplasm growing as "Rosette" that is histochemically positive for neuron specific enolase (NSE). ,,
| Case Report|| |
This was a case report of a 24-year-old male patient who was admitted in the chest department with pain in the left side of chest for a period of about 3 months, dyspnea for about 2 months and lump at left upper part of the abdomen for 1 month; which were accompanied by loss of appetite and weakness and weight loss (about 3.0-4.0 kg). On examination, he had mild pallor, no palpable lymph node and blood pressure was normal (126/72 mmHg).
Examination of the chest revealed left sided massive pleural effusion. On examination of abdomen, liver and spleen were not palpable, a tender lump was found at left hypochondrium, which was extended up to 5 cm below the costal margin; other systems were within the normal limit.
Chest X-ray [Figure 1] was suggestive of left sided massive pleural effusion with mediastinal shift to the opposite side. Routine investigations such as fasting blood sugar (108 mg%), serum creatinine (0.9 mg%), blood urea nitrogen (18 mg%) and serum electrolytes were within normal limit except hemoglobin (9.5 g%). Human immunodeficiency virus test was non-reactive. 24 h urinary catecholamine metabolite (vanillyl mandelic acid) was slightly raised. About 3.2 L of hemorrhagic fluid were drained through intercostal chest tube drainage. It was an exudative effusion (total protein-4.5 g%, sugar-76 mg%, ADA-15.4 IU/Lt, total cells-100/cu.mm, N-7%, L-90%, mesothelial cells-3%, Gram stain and ZN stain were negative and polymerase chain reaction for mycobacteria tuberculosis complex was negative). Pleural fluid cytology showed a tight cluster of small round cells [Figure 2]. On ultrasonography (USG) of abdomen-a large well-defined heterogenous mass lesion (size 15.5 cm × 11 cm) in left adrenal region was detected. All the other abdominal organs were normal in size, shape, position and echotexture; there was no free fluid in the peritoneal cavity and no lymphadenopathy was detected.
|Figure 1: Chest X-ray P-A view showing left sided massive pleural effusion|
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|Figure 2: Photomicrograph of pleural fluid cytology showing tight cluster of small round cells|
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Computed tomography (CT) of abdomen [Figure 3]. There was no free fluid in the peritoneal cavity, no lymphadenopathy and normal retroperitoneal fat planes. All the organs were normal and without any focal lesion, except left adrenal gland, where a huge heterogeneously enhancing mass lesion was seen with areas of necrosis that extends superiorly to left thorax causing compression of the left lower lobe of the lung. Under CT guidance, fine-needle aspiration was done from the mass. Cytological examination of aspirated material suggested Small round cell tumor with the formation of "Rosette" [Figure 4]. Immunohistochemical stains for NSE in cell block preparation was positive, which established the diagnosis of neuroblastoma. CT-scan thorax-was carried out after drainage of pleural fluid from left pleural space and reported that there is minimal fluid on the left pleural space and compression of the left lower lobe of lung by the huge mass extended from abdomen, but upper lobe appeared normal; on the right side-lung parenchyma, pleura and hilum were normal. Mediastinal structures were normal but shifted to the right side.
|Figure 3: Computed tomography-scan abdomen showing heterogeneous mass at left adrenal region|
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|Figure 4: Photomicrograph of fine-needle aspiration of left adrenal mass showing Rosette|
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| Discussion|| |
Here, we present a case of adrenal neuroblastoma in adult male which is extremely rare. This case presented with left sided massive pleural effusion and lump at left hypochondrium. Imaging revealed heterogeneously enhancing mass originating from left adrenal gland area; with extension of mass to the left lower chest producing collapse of the left lower lobe and left sided massive pleural effusion. On laboratory investigations, 24 h urinary catecholamine metabolite (vanillyl mandelic acid) was raised and it is found in more than 90% of patients.  Cytological examination of pleural fluid and fine-needle aspiration cytology of mass lesion revealed that the tumor is a small round cell type with "Rosette" formation; and histochemically it is positive. All these findings confirmed that the tumor is adrenal neuroblastoma.
Pleural effusion is uncommon at diagnosis of neuroblastoma. No definite reports regarding the prevalence of pleural effusion were found, due to its rarity. However, presence of pleural effusion with positive malignant cells (stage 4) significantly changes the management and outcome of the neuroblastoma.
This scan uses a form of chemical meta-iodobenzyl guanidine (MIBG) that contains a small amount of radioactive iodine. After injection of this chemical, it will attach to neuroblastoma cells anywhere in the body. Then several hours later the body is scanned with a special camera to look for areas that picked up the activity-indicates the diagnosis of neuroblastoma, about its location and spread to other parts or not, also used after treatment to see remaining tumor or monitoring the treatment response.
Treatment varies depending on-the location of the tumor, how much and where the tumor has spread and the patients' age. More than one of these treatment modalities may be used, depending on the stage of the disease. The four types of treatment modalities are used (1) surgery, (2) radiation therapy, (3) chemotherapy (cisplatin, doxorubicin, cyclophosphamide, etoposide, teniposide) and (4) bone marrow transplantation.
As per International Neuroblastoma Staging System: Defining the disease: (1) Low risk (stage 1 and stage 2), (2) intermediate-risk (stage 3 and stage 4 with age <18 months, stage 4S) and (3) high risk disease (stage 4 metastatic disease).
Low risk disease
Age less than 1 year at diagnosis or small tumor-surgery and follow-up.
Larger tumor that has not spread or has spread (but does not have the MYCN abnormalities) - combined surgery and chemotherapy (4-8 months).
High risk disease
Half of patients have a high risk disease either spread to other parts of the body or tests result show MYCN amplification-combination of chemotherapy, surgery and radiation therapy. Chemotherapy is followed by his/her stem cell transplantation.
The chances of recovery from neuroblastoma depends on the stage of the cancer, the age of the patient at diagnosis, the location of the tumor; and the state and nature of the tumor cells evaluated under the microscope. Infants have a higher rate of cure than do children over 1 year of age, even when the disease has spread. In very young children with neuroblastoma, the tumor may go away on its own, without any treatment or the tissues of the tumor may mature and develop into a non-cancerous (benign) tumor called a ganglioneuroma, which can be surgically removed. After treatment follow-up tests (MIBG scan, positron emission tomography scan, USG, CT scan/magnetic resonance imaging scan) to see if there is any tumor remaining. Test depends on risk groups, tumor size and location needs long-term follow-up. High risk neuroblastoma has very high rate of relapse/recurrence. The predicted 5 years survival rate is approximately 85% for children who had the onset of disease in infancy and 35% for those whose disease developed later.
| References|| |
|1.||Yapanoðlu T, Ӧzbey I, Aksoy Y, Gürsan N, Polat Ӧ. Neuroblastoma in adulthood. Turk J Med Sci 2008;38:281-3. |
|2.||Lacayo NJ, Coppes MJ. Pediatric neuroblastoma. Medscape Reference, 2012. Available from: http://www.emedicine.medscape.com/article/988284-overview#a0104. [Last accessed Oct, 2013] |
|3.||Redman JL, Agerty HA, Barthmaier OF, Fisher HR. Adrenal neuroblastoma report of a case and review of the literature. Arch Pediatr Adolesc Med 1938;56:1097-112. |
|4.||Genc H, Haciyanli M, Haciyanli SG, Gelal F, Avci Uçarsoy A, Dag F. An adult adrenal neuroblastoma: A case report. Acta Chir Belg 2005;105:673-6. |
|5.||Rajwanshi A, Srinivas R, Upasana G. Malignant small round cell tumors. J Cytol 2009;26:1-10. |
|6.||Genc FA, Aksoy M, Kapran Y, Tunca F, Tanakol R, Basar Y, et al. Adrenal neuroblastoma in an adult: Report of a case. Surg Today 2003;33:879-81. |
|7.||Refaat MM, Idriss SZ, Blaszkowsky LS. Case report: An unusual case of adrenal neuroblastoma in pregnancy. Oncologist 2008;13:152-6. |
|8.||Yamamoto K, Hanada R, Kikuchi A, Ichikawa M, Aihara T, Oguma E, et al. Spontaneous regression of localized neuroblastoma detected by mass screening. J Clin Oncol 1998;16:1265-9. |
|9.||Gann DS, Demaria EJ. Adrenal. In: Schwartz SI, Shires TG, Spencer FC, editors. Principles of Surgery. 6 th ed. New York: McGraw-Hill; 1994. p. 1561-09. |
|10.|| Meltzer S. Neuroblastoma occurring in adults. Can Med Assoc J 1926;16:647-51. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]