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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 614-615
Ganglioneuroblastoma presenting as a subcutaneous lump diagnosed by fine needle aspiration


Department of Pathology, Lady Hardinge Medical College, New Delhi, India

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Date of Web Publication20-Sep-2011
 

How to cite this article:
Choudhury M, Singh S, Goel RK. Ganglioneuroblastoma presenting as a subcutaneous lump diagnosed by fine needle aspiration. Indian J Pathol Microbiol 2011;54:614-5

How to cite this URL:
Choudhury M, Singh S, Goel RK. Ganglioneuroblastoma presenting as a subcutaneous lump diagnosed by fine needle aspiration. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Nov 29];54:614-5. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/614/85115


Ganglioneuroblastoma (GNB) is a rare, neuroblastic tumor arising from the sympathetic nervous system. In the literature, reports of this entity, diagnosed by fine needle aspiration and its cytological appearance are scarce. A three-year-old girl presented to the pediatric surgery outpatient department with a subcutaneous lump of one year duration with urinary and bowel incontinence and progressive inability to walk. On examination, there was a firm, non-tender mass measuring 4 3 cm in the left lumbar and paravertebral region [Figure 1]a. Routine laboratory tests were within normal limits. Contrast-enhanced computed tomography (CECT) showed an ill-defined heterogeneous enhancing soft tissue density lesion 6 4.2 3.6 cm in the left paravertebral region with peripheral calcification. The lesion was extending from the left kidney to the level of the pelvic brim with involvement of the erector spinae and quadrates lumborum muscles with intraspinal extension [Figure 1]b. Fine needle aspiration (FNA) of the mass was performed using a 22-G needle. Smears were stained with Papanicolaou, hematoxylin and eosin (H and E), and Giemsa stain. Aspirates were cellular with a fine fibrillary background. Two different cell populations were observed; the predominant type comprised large, oval to polygonal cells with abundant granular cytoplasm. The nuclei were one to three in number, round, vesicular, eccentrically located with prominent nucleolus. The other cells were small round cells with scant cytoplasm, having irregular hyperchromatic nuclei. These cells were few in number and formed rosette-like structures [Figure 2]. A diagnosis of GNB was made. The diagnosis was supported by H and E sections of cell block preparation of aspirated material. Tumor cells also showed uniform positivity for nonspecific esterase and chromogranin A on immunohistochemistry [Figure 2] inset. The diagnosis of GNB was confirmed. Further clinical course of the patient could not be assessed as the patient was lost to follow-up.
Figure 1: (a) Subcutaneous lump in the left lumbar and paravertebral region, (b) CECT abdomen showing ill-defined, heterogeneous, enhancing soft tissue lesion in the left paravertebral region with intraspinal extension

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Figure 2: Small round cells with scant cytoplasm admixed with ganglion cells (pap; x400) with inset showing chromogranin positivity (IHC, x100)

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   Discussion Top


GNB is also known as a transitional tumor, originating from the neural crest sympathgonia, which are completely undifferentiated cells of the sympathetic nervous system. They contain primitive neuroblast and mature ganglion cells and are considered to have an intermediate potential for malignancy as compared to benign ganglioneuroma and malignant neuroblastoma. [1] GNBs occur most commonly in children and are usually found in the adrenal medulla, retroperitoneum, posterior mediastinum, and head and neck region. [2],[3] The most common presentation is abdominal distension, pain and neurologic symptoms due to nerve root compression and Horner's syndrome. Clinical presentation of GNB as a subcutaneous mass is unusual. FNA may not yield either of the two components (ganglioneuromatous and neuroblastomatous) leading to incorrect diagnosis of ganglioneuroma (GN) and neuroblastoma. Therefore, these tumors should be aspirated from multiple sites and thoroughly screened before giving a final diagnosis. Ancillary procedures such as cell block preparation and immunohistochemistry also contribute to an accurate diagnosis. Proliferative fasciitis and myositis may also show ganglion-like cells. However, sheets of closely packed plump spindle cells, multinucleated muscle fibers are seen in proliferative myositis in the absence of a neuroblastomatous component. [4] Moreover, the clinical course and immune profile of ganglion-like cells help in differentiating these lesions from GNB. GNB rarely presents as a subcutaneous lump. Moreover, the present case also highlights the importance of FNA as a rapid, cost-effective and safe modality in the diagnosis of this tumor and differentiates it from other benign and malignant neural tumors. A definite cytological diagnosis is mandatory in the preoperative diagnostic workup and for precise pre-surgical chemotherapy.

 
   References Top

1.Ambros PF, Ambros IM, Brodeur GM, Haber M, Khan J, Nakagawara A, et al. International consensus for neuroblastoma molecular diagnostics: Report from International Neuroblastoma Risk group (INRG) Biology Committee. Br J Cancer 2009;100:1471-82.  Back to cited text no. 1
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2.Adam A, Hochhholzer L. Ganglioneuroblastoma of the posterior mediastinum: A clinicopathologic review of 80 cases. Cancer 1981;47:373-81.  Back to cited text no. 2
    
3.Moukheiber AK, Nicollas R, Roman S, Coze C, Triglia JM. Primary pediatric neuroblastic tumors of the neck. Int J Pediatr Otorhinolaryngol 2001;60:155-61.  Back to cited text no. 3
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4.Wong NL. Fine needle aspiration cytology of pseudosarcomatous reactive proliferative lesions of soft tissue. Acta Cytol 2002;46:1049-55.  Back to cited text no. 4
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Correspondence Address:
Smita Singh
Department of Pathology, A-207, Narwana Apartments, Plot No. 89, I.P. Extension, Delhi - 110 092
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.85115

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