Journal of Cytology
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CASE REPORT  
Year : 2013  |  Volume : 30  |  Issue : 4  |  Page : 278-279
Loose cell clusters with vascular coats: Zellballen pattern of paraganglioma on cytology


1 Department of Pathology, BYL Nair Ch. Hospital & T N Medical College, Mumbai, Maharashtra, India
2 Department of Surgery, BYL Nair Ch. Hospital & T N Medical College, Mumbai, Maharashtra, India

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Date of Web Publication6-Feb-2014
 

   Abstract 

Paragangliomas are uncommon tumors arising in paraganglion tissue, which is well represented in the head and neck. A correct pre-operative cytological diagnosis helps in planning early and optimal surgery. Cytologic features in carotid body tumor and extra adrenal paragangliomas have been well described and documented. In addition to the acinar and discrete arrangement of cells, smears also showed a few large loose clusters of cells enveloped by thin vascular channels-the so-called zellballen pattern, a diagnostic feature on histopathology. Zellballen pattern of paraganglioma on cytology is not a common finding and hence is being reported.

Keywords: Carotid body tumor; cell clusters enveloped by vessels; cytological features; paraganglioma; vascular coat; zellballen pattern

How to cite this article:
Jashnani KD, Patil RD, Balsarkar DJ. Loose cell clusters with vascular coats: Zellballen pattern of paraganglioma on cytology. J Cytol 2013;30:278-9

How to cite this URL:
Jashnani KD, Patil RD, Balsarkar DJ. Loose cell clusters with vascular coats: Zellballen pattern of paraganglioma on cytology. J Cytol [serial online] 2013 [cited 2018 Nov 20];30:278-9. Available from: http://www.jcytol.org/text.asp?2013/30/4/278/126670



   Introduction Top


There are many reports of paraganglioma diagnosed on fine needle aspiration cytology (FNAC) in the literature. Although FNAC of a suspected paraganglioma is contraindicated in view of the hypertensive crisis after fine-needle aspiration, this tumor can be an unexpected finding in neck mass FNACs performed in unsuspecting cases. Cytologic features of single cell, loose clusters and occasional acinar configuration of large, oval cells with round to oval nuclei, evenly dispersed chromatin, focally discrete nucleoli, moderate anisocytosis and anisonucleosis have been well described for paragangliomas. [1],[2],[3] In the present case in addition to the acinar and discrete arrangement of cells, smears also showed few large loose clusters of cells enveloped by thin vascular channels the so called zellballen pattern, a diagnostic feature on histopathology. Hence it is being documented.


   Case Report Top


We describe a case of a 42-year-old woman presenting with fever on and off since 1 year and neck swelling of 5 months duration. Ultrasonography (USG) showed a well-defined heterogeneous highly vascular lesion in right cervical region. Thyroid, parotid, submandibular gland and neck vessels were normal. Clinical impression was lymph node metastasis or a carotid body tumor. Clinical examination revealed a solitary 3 cm × 2 cm non-mobile non-tender mass present at the right angle of mandible. FNAC was bloody and Pap stained smears revealed cells in clusters, microacinar and rosette like formations as well as singly. The cells were monomorphic, round to plasmacytoid with round to oval nuclei and a moderate amount of pale granular cytoplasm. There was evidence of mild anisonucleosis. Many of the cells showed stippled nuclear chromatin. There were in addition, few large loose clusters of cells enveloped by vascular channels on all sides. The cells within these clusters were arranged in curved rows. These cells showed same cytomorphologic features as surrounding cells [Figure 1]a and b. There was no evidence of necrosis or mucin in the smears.
Figure 1: (a) Cells arranged in compact clusters surrounded by vascular channels on all sides (Pap, ×100). (b) Endothelial cells in vascular channel enveloping tumor cells on one surface of the same cluster seen in (a) (Pap, ×200). (c) Typical zellballen pattern on histopathology of the same case (H and E, ×100)

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In view of monomorphic cells with the absence of necrosis and mucin, metastatic adenocarcinoma was ruled out. Metastasis of medullary carcinoma of the thyroid was considered on cytomorphology, but was ruled out on the basis of a normal thyroid on USG and absence of specific cytologic features. Hence a cytological diagnosis of carotid body paraganglioma was offered. After 2 weeks, the patient underwent surgical excision with due precautions. Histopathological examination confirmed the diagnosis of a benign carotid body paraganglioma [Figure 1]c.


   Discussion Top


This case report highlights the presence of loose cell clusters enveloped by thin vascular channels on cytology, a feature which has been occasionally described in anecdotal case reports [4],[5] in intraoperative scrape smears. This cytologic pattern corresponds with the diagnostic zellballen pattern described on histopathology. The presence of cell clusters with vascular coats on cytology should lead to high index of suspicion for pheochromocytoma/paraganglioma (adrenal or extra adrenal) in addition to the usual cytological, clinical and radiological features. It is important to diagnose this condition pre-operatively in order to avoid any surgical delay. Clusters and discretely scattered cells, with occasional acinar configuration and fine reddish intracytoplasmic granules are the established facts of a paraganglioma. However cell clusters enveloped by thin vascular channels on cytology the so called zellballen pattern diagnostic of histopathology may be considered as a novel insight and should be sought for in cytology smears when paraganglioma is a high suspicion.

 
   References Top

1.Rana RS, Dey P, Das A. Fine needle aspiration (FNA) cytology of extra-adrenal paragangliomas. Cytopathology 1997;8:108-13.  Back to cited text no. 1
    
2.Varma K, Jain S, Mandal S. Cytomorphologic spectrum in paraganglioma. Acta Cytol 2008;52:549-56.  Back to cited text no. 2
    
3.Gong Y, DeFrias DV, Nayar R. Pitfalls in fine needle aspiration cytology of extraadrenal paraganglioma. A report of 2 cases. Acta Cytol 2003;47:1082-6.  Back to cited text no. 3
    
4.Han JY, Lee KG. Fine needle aspiration cytology of paraganglioma of retroperitoneum. Korean J Cytopathol 1992;3:19-24.  Back to cited text no. 4
    
5.Shidham VB, Galindo LM. Pheochromocytoma. Cytologic findings on intraoperative scrape smears in five cases. Acta Cytol 1999;43:207-13.  Back to cited text no. 5
    

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Correspondence Address:
Kusum D Jashnani
8, Aashirvad Building, First Floor, Opposite Kakad Industrial Estate, Lady Jamshedji Cross Road No. 3, Mahim, Mumbai - 400 016, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9371.126670

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