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CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 5  |  Page : 507-509
Chondroid syringoma of the forearm: A case report of a rare localization


1 Department of Pathology, Razi Hospital, Guilan University of Medical Sciences, Rasht, Iran
2 Student Research Committee, Guilan University of Medical Sciences, Rasht, Iran
3 Pars Clinic, Rasht, Iran

Date of Web Publication1-Sep-2014

Correspondence Address:
Seyedeh Nazanin Seyed Saadat
Research Vice Chancellorship Building, Imam Khomeini Blvd., Rasht, Gilan
Iran
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.139914

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   Abstract 

Chondroid syringoma (CS) is an uncommon benign adnexal tumor of the skin with eccrine and apocrine origin, which usually involves the head and neck region. The presentation of CS in other areas of the body is rare. A 45-year-old male patient presented to the dermatology clinic with a chief complaint of a painless, slow-growing mass on his left forearm, which gradually developed over the course of 2 years. A solitary, firm, purple, mobile, non-tender nodule was located in the distal part of left dorsal forearm, which was 1.8 cm in diameter. The tumor was surgically excised and sent for the histopathological evaluation. Results of biopsy and hematoxylin and eosin staining confirmed the diagnosis of CS and showed no evidence of malignancy. Although CS is an uncommon tumor in uppr limb region, it should be considered as one of the differential diagnoses, when dealing with tumors of this area.


Keywords: Adenoma, forearm, pleomorphic, syringoma


How to cite this article:
Askari K, Ghorbani G, Yousefi N, Saadat S, Saadat SS, Zargari O. Chondroid syringoma of the forearm: A case report of a rare localization. Indian J Dermatol 2014;59:507-9

How to cite this URL:
Askari K, Ghorbani G, Yousefi N, Saadat S, Saadat SS, Zargari O. Chondroid syringoma of the forearm: A case report of a rare localization. Indian J Dermatol [serial online] 2014 [cited 2019 Jun 17];59:507-9. Available from: http://www.e-ijd.org/text.asp?2014/59/5/507/139914

What was known?
Chondroid syringoma is a rare benign adnexal tumor which typically appears over the head and neck region.



   Introduction Top


Chondroid syringoma (CS) is an uncommon benign adnexal tumor of the skin with eccrine and apocrine origin, which usually involves the head and neck region. [1] The presentation of CS in other areas of the body is unusual. [2] Due to the presence of both epithelial and mesenchymal components, CS was used to be formerly called as a mixed tumor of the skin. [3]

Due to non-specific presentation, diagnosis is usually made by histopathologic studies. [4] However, CS can be diagnosed using fine needle aspiration cytology. [3] Total surgical excision is the mainstay of treatment for benign CS. Although CS is a benign tumor, malignant form has rarely been reported; therefore, patients are recommended to have regular follow-up after surgery. [1]

CS is uncommon and usually arises in head and neck region. Involvement of other parts of the body is very unusual for this uncommon tumor. [5] Herein, we report a rare case of a CS located over the forearm of a 45-year-old man.


   Case Report Top


This was a case report of a 45-year-old male patient presented to the dermatology clinic complaining of a painless, slow-growing mass on his left forearm, which gradually developed over the course of 2 years [Figure 1]. Patient reported no allergies and did not smoke or take any specific medications. On physical examination, there was a solitary, firm, purple, mobile, non-tender nodule located in the distal part of his left dorsal forearm, which was 1.8 cm in diameter. There were no palpable regional lymph nodes. Laboratory studies, including complete blood count, erythrocyte sedimentation rate and liver function tests were within normal limits. With the possible diagnoses of pilomatricoma, dermatofibrosarcoma and adnexal tumors in mind, the tumor was surgically excised and sent for the histopathological evaluation.
Figure 1: A firm, painless, mobile nodule located on the left forearm

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Macroscopic examination showed a well-circumscribed tumor with an epithelial component in a myxoid, chondroid and hyalinized fibrous stroma [Figure 2]. The epithelial component was composed of clusters and solid cords of cells as well as ductal structures with occasional branching lined by two layers of cuboid cells. Solid islands of squamous epithelial cells as well as hyaline epithelial cells with plasmacytoid appearance were present [Figure 2] and [Figure 3]. The immunohistochemical study was positive for cytokeratin, vimentin and S-100 protein in the stroma. Results of biopsy and hematoxylin and eosin staining confirmed the diagnosis of CS and showed no evidence of malignancy.
Figure 2: A nodular tumor comprised of small aggregates of epithelial cells without lumina and single epithelial cells embedded in abundant chondroid, myxoid and hyalinized stroma (H and E, ×10). Insert: Abundant stroma shows highly hyalinized fibroma changes with chondromyxoid pattern (H and E, ×40)

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Figure 3: Rare tubular lumina are lined by two layers of epithelial cells. Cells in the mucoid stroma show the appearance of chondrocytes

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   Discussion Top


CS is an uncommon mixed tumor of the skin that was first described by Billorth in 1859. [2],[6] CS generally presents as a painless, slow-growing and asymptomatic nodule in the head and neck region, which most often affects elderly and middle aged men. [1],[2],[4],[7] Most CS are well-circumscribed subcutaneous nodules measuring 0.5-3 cm in diameter. [1],[2],[8] CS is usually arises in head and neck region. Involvement of other parts of the body is very unusual for this uncommon tumor. [2]

CS is a benign lesion in most of the cases and recurrence is uncommon; [9] however, malignant forms of CS has rarely been reported. [7],[8] Malignant forms of CS are more common in women and predominantly affect trunk and extremities.  [1],[2],[8] Malignant tumors are usually larger than 3 cm in size; however, benign tumor measured 10 cm may also be present. [10]

CS does not have characteristic diagnostic clinical features, thus the diagnosis of CS is made by histopathologic studies. [4],[8] Important considerations in differential diagnosis of CS are neurofibroma, dermatofibroma, pilomatricoma, histiocytoma, seborrheic keratosis, sebaceous cyst, basal cell carcinoma and squamous cell carcinoma. [2],[7],[8]

Histologically, a wide variety of structures may be found in CS of skin. Most of them have a cartilage like matrix with varying epithelial structures distributed among myxoid, chondroid and fibrous stroma. [1],[2] The epithelial component includes clusters and solid cords of cells as well as ductal structures with occasional branching mostly lined by two layers of cuboidal cells, sometimes ducts are dilated with keratinous cysts formation. [5] CShas apocrine and eccrine variants. [1] The eccrine type is composed of small lumens, which are lined by a row of cuboidal epithelial cells. The apocrine type is formed from cystic and tubular branching lumen and is covered with two rows of epithelial cells.  [1],[2],[8] Eosinophilic globules composed of collagen (so-called collagenous spherulosis) are rarely present and occasionally focal calcifications can be seen. In few cases, the mesenchymal component shows plasmacytoid appearance due to peripheral displacement of the nuclei, which is called hyaline-cell rich CS, [5] as the case we presented herein.

Total surgical excision is the treatment of choice for patients with benign CS. [1],[2],[8] The risk of malignancy of this tumor is extremely rare, but it has been reported and therefore patients are recommended to have regular follow-ups. [1],[2],[8] Our patient was treated by complete excision and had no recurrence within 6 months follow-up.


   Conclusion Top


Although CS is an uncommon tumor in upper limb region, it should be considered as one of the differential diagnoses, when dealing with tumors of this area. Regular follow-up of the CS patient is recommended to evaluate the risk of malignancy and local recurrence.

 
   References Top

1.Solanki LS, Dhingra M, Bhalla M, Thami GP, Punia RP, Batra S. Chondroid syringoma: Report of two cases in young patients. Dermatol Online J 2011;17:7.  Back to cited text no. 1
    
2.Mebazaa A, Trabelsi S, Denguezli M, Sriha B, Belajouza C, Nouira R. Chondroid syringoma of the arm: An unusual localization. Dermatol Online J 2006;12:14.  Back to cited text no. 2
    
3.Kumar B. Chondroid syringoma diagnosed by fine needle aspiration cytology. Diagn Cytopathol 2010;38:38-40.  Back to cited text no. 3
[PUBMED]    
4.Paul K, Sreekar H, Dhanraj P, Lamba S, George SM. Chondroid syringoma with extensive ossification. Ann Maxillofac Surg 2011;1:91-2.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.Weedon D. Tumors of cutaneous appendages. In: Weedon D, editor. Skin Pathology. 2 nd ed. Edinburgh: Churchill Livingstone; 2002. p. 881.  Back to cited text no. 5
    
6.Belfquih H, El Mostarchid B, Oukabli M, Akhaddar A, Boucetta M. Benign chondroid syringoma of the orbit: A rare cause of exophtalmos. Head Face Med 2012;8:8.  Back to cited text no. 6
    
7.Vasileiadis I, Kapetanakis S, Petousis A, Karakostas E, Simantirakis C. Rapidly growing chondroid syringoma of the external auditory canal: Report of a rare case. Case Rep Med 2011;2011:589680.  Back to cited text no. 7
    
8.Sirivella S, Gielchinsky I. Chondroid syringoma: A rare tumor of the chest wall. Ann Thorac Surg 2010;89:983-5.  Back to cited text no. 8
    
9.Ramaswamy AS, Yenni VV, Wilfred C, Manjunatha HK, Shilpa K. Hyaline cell-rich chondroid syringoma of the finger. Indian J Dermatol 2011;56:217-9.  Back to cited text no. 9
[PUBMED]  Medknow Journal  
10.Uyar B, Solak A, Sahin N, Bugdayci H. Giant chondroid syringoma radiologically mimicking malignancy. Indian J Dermatol 2013;58:245.  Back to cited text no. 10
[PUBMED]  Medknow Journal  

What is new?
A case of chondroid syringoma affected the left forearm is reported here on account of its unusual localization.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    Abstract
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