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E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 245
Giant chondroid syringoma radiologically mimicking malignancy


1 Department of Dermatology, Sifa University, Fevzipasa Bulvar? Basmane, Izmir, Turkey
2 Department of Radiology, Sifa University, Fevzipasa Bulvar? Basmane, Izmir, Turkey
3 Department of Pathology, Sifa University, Fevzipasa Bulvar? Basmane, Izmir, Turkey

Date of Web Publication20-Apr-2013

Correspondence Address:
Belkiz Uyar
Department of Dermatology, 35240, 172/2 Fevzipasa Bulvar? Basmane, Izmir
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.110879

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   Abstract 

Chondroid syringoma, or mixed tumor of skin, is a relatively rare, usually benign sweat gland tumor, most often seen in the head-and-neck region. Rare malignant examples have been reported, commonly involving the extremities. We report here a case radiologically mimicking a malignant neoplasm, but histologically-proven benign subcutaneous chondroid syringoma, arising in the anterior aspect of the upper thigh of a 59-year-old male.


Keywords: Chondroid syringoma, magnetic resonance images, sweat gland tumor


How to cite this article:
Uyar B, Solak A, Sahin N, Bugdayci H. Giant chondroid syringoma radiologically mimicking malignancy. Indian J Dermatol 2013;58:245

How to cite this URL:
Uyar B, Solak A, Sahin N, Bugdayci H. Giant chondroid syringoma radiologically mimicking malignancy. Indian J Dermatol [serial online] 2013 [cited 2019 Oct 14];58:245. Available from: http://www.e-ijd.org/text.asp?2013/58/3/245/110879

What was known? Cases of chondroid syringoma evaluated by conventional magnetic resonance imaging are described



   Introduction Top


Chondroid syringoma is a relatively rare, benign, skin appendage tumor. The head and neck regions are common sites where intra-dermal or subcutaneous firm nodules of 0.5-3 cm are found. [1]

Although its histological findings are well-described, the radiological features of chondroid syringoma have been reported in only few cases.


   Case Report Top


A 59-year-old man presented with a painless mass on the anterior aspect of the upper thigh with a slow enlargement over the past 5 years [Figure 1]. Dermatological examination showed a relatively firm, mobile 5 × 3 cm nodule covered by normal skin. The patient was otherwise healthy. Laboratory studies including complete blood count, erythrocyte sedimentation rate (ESR), urine analysis, aspartate aminotransferase (AST), alanine aminotransferase (ALT), blood urea, creatinine were normal. The findings were suggestive of lipoma or liposarcoma. Sonographic examination revealed mixed, echogenic solid mass measuring approximately 4.5 × 3 × 2 cm. On Doppler ultrasound, there were vessels predominantly at the periphery of the mass. Magnetic resonance images (MRI) revealed a 4.7 × 2.9 × 2.2 cm, well-circumscribed, lobular mass that showed heterogeneous enhancement predominantly in the peripheral portion of the mass after intravenous contrast administration [Figure 2]. Diffusion-weighted imaging (DWI) showed areas of increased signal intensity that were indicative of a highly cellular tumor [Figure 3]. According to the MRI findings, we considered benign subcutaneous lipomatous tumors with cystic and solid components such as angiolipoma or fibrolipoma or well-circumscribed low-grade soft tissue liposarcoma without surrounding tissue extension in our differential diagnosis. Because the vascularity of the lesion and heterogeneous pattern with contrast enhancement on MRI examination, we excluded lipoma.
Figure 1: Relatively firm, 5 × 3 cm, lipoma-like mass covered by normal skin

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Figure 2: Post-contrast axial fat-saturated T1-weighted images show well-circumscribed, lobular mass with heterogeneous enhancement

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Figure 3: Diffusion-weighted imaging (b: 800) signs of increased restriction in diffusion, a finding indicative of a highly cellular tumor

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We decided to perform trucut biopsy. Histopathological examination revealed a group of cells, which have eosinophilic cytoplasm and regular small nuclei, exhibited epithelial-like pattern in the myxoid stroma. Many of the properties were lipomatous. However, as adipocytes were variable in size and focally showed a lipoblast-like appearance, some tumor areas were reminiscent of a well-differentiated lipoma-like liposarcoma [Figure 4].
Figure 4: Tru-cut biopsy. Microscopic examination shows a focal area of lipoblast-like appearance in the myxoid background (H and E, ×10)

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The patient underwent surgical excision of the mass. Histologically, the tumor was confined to the subcutaneous tissue. The adjacent tissue appeared normal. There were irregular epithelial nests and tubuloalveolar structures in a chondromyxoid stroma. There was also abundant fibrolipomatous stroma. There were foreign body reactions against the epidermoid cysts within the tumor and surrounding tissue. There were no atypical mitoses or necrosis [Figure 5]a and b.
Figure 5

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   Discussion Top


Chondroid syringoma is a relatively rare, benign, skin appendageal tumor. The head and neck regions are common sites; less commonly, axillary region, hand, foot, abdomen, penis, vulva, scrotum can be involved. [1],[2]

Chondroid syringoma usually presents as a solitary, asymptomatic, intracutaneous or subcutaneous nodule, mainly seen in middle-aged or older males. [2],[3] The size is usually between 0.5 and 3 cm, but it may be very large. [2],[3] Most chondroid syringomas are benign with a slow progression over years. [4] In our case, the mass enlarged slowly over 5 years. Malignancy in CS is rare. [4],[5] Malignant lesions are usually larger than 3 cm, but benign one measuring 10 cm is also reported. [4]

Histologically, a wide variety of structures may be found in chondroid syringoma of skin. Eccrine types have multiple small syringoid elements and are rich in mucin. In the apocrine type, lesions have follicular or sebaceous elements as well as larger dilated branching glanduler elements. The mesenchymal component can show chondroid differentiation or even metaplastic ossification. [6] In our case, there was abundant lipomatous stroma that made diagnostic difficulty in radiological imaging and histopathological examination.

Previous case reports have described the MR imaging findings of benign and malignant chondroid syringomas. [7],[8],[9] Although the MR imaging appearance of chondroid syringoma is relatively non-specific, MR imaging generally may allow characterization of the origin of tissue components in the tumor and depiction of the extent and relationships of the lesion to adjacent structures. In our case, the signal intensity characteristics of the lesion (heterogeneous lesion of mixed fat and fluid-like intensity), while not specific for chondroid syringoma, allowed for some analysis of the consistency of the tumor.

The benign neoplasms of the subcutaneous tissue that may have similar appearances with our case include superficial lipoma and variants of lipoma (angiolipoma, myolipoma, spindle-cell and pleomorphic lipoma, chondroid lipoma, and hibernoma). Although subcutaneous in location, superficial lipoma consists entirely of fat, unlike our lesion. Variants of lipoma are commonly heterogeneous and include more than one type of tissue, which may be fat, muscle, chondroid matrix, vascular elements, or myxoid tissue with calcification and hemorrhage. [4],[10] Due to the presence of vascular channels on Doppler examination, angiolipoma was considered in our case.

The malignant tumors that may bear similar characteristics with mixed tumor of the skin include well-differentiated liposarcoma, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, myxofibrosarcoma, and malignant peripheral nerve sheath tumors. [10]

After tru-cut biopsy, we considered many of the above entities in the differential diagnosis. But excisional biopsy allowed definitive diagnosis of chondroid syringoma based on the morphological features. There were no cytologic atypia, increased mitotic figures, tumor necrosis, or infiltrative margins, thus excluding malignant chondroid syringoma.

The mixed fat and fluid-like signal intensity lesion of skin is somewhat of a diagnostic enigma due to its non-specific imaging and histologic presentation. Clearly a soft tissue lesion of mixed fat and fluid-like signal intensity, as in this case, while not pathognomonic for chondroid syringoma, allows for a limited differential diagnosis. As this case shows, chondroid syringoma should be included in the differential diagnosis of a slowly growing, nodular heterogeneous mass in the skin or subcutaneous tissue. Final diagnosis, however, requires careful histopathologic examination.

 
   References Top

1.Hirsch P, Helwig EB. Chondroid syringoma. Mixed tumor of skin, salivary gland type. Arch Dermatol 1961;84:835-47.  Back to cited text no. 1
    
2.Yavuzer R, Baºterzi Y, Sari A, Bir F, Sezer C. Chondroid syringoma: A diagnosis more frequent than expected. Dermatol Surg 2003;29:179-81.  Back to cited text no. 2
    
3.Villalón G, Monteagudo C, Martín JM, Ramón D, Alonso V, Jordá E. Chondroid syringoma: A clinical and histological review of eight cases. Actas Dermosifiliogr 2006;97:573-7.  Back to cited text no. 3
    
4.Sungur N, Uysal A, Gümüº M, Koçer U. An unusual chondroid syringoma. Dermatol Surg 2003;29:977-9.  Back to cited text no. 4
    
5.Barnett MD, Wallack MK, Zuretti A, Mesia L, Emery RS, Berson AM. Recurrent malignant chondroid syringoma of the foot: A case report and review of the literature. Am J Clin Oncol 2000;23:227-32.  Back to cited text no. 5
    
6.Rütten A. Adnexal tumors. In: Braun-Falco O, Burgdorf WH, Plewig G, Wolff HH, Landthaler M, editors. Braun Falco's Dermatology. 3 rd ed. Heidelberg, Italya: Springer Medizin Verlag; 2009. p. 1377-96.  Back to cited text no. 6
    
7.Kerimoglu U, Aydingoz U, Ozkaya O, Aksu AE, Ergen FB. MRI of a benign chondroid syringoma. Br J Radiol 2006;79:e59-61.  Back to cited text no. 7
    
8.Park HJ, Shin KH, Yang WI, Sun JS. Growing mass on the medial aspect of the right proximal leg. Clin Orthop Rel Res 2000;380:279-83.  Back to cited text no. 8
    
9.Kakitsubata Y, Theodorou SJ, Theodorou DJ, Nakahara M, Yuki Y, Yokouchi T. Giant chondroid syringoma presenting as a growing subcutaneous mass in the upper arm: MRI findings with pathologic correlation. Joint Bone Spine 2009;76:711-4.  Back to cited text no. 9
    
10.Peterson JJ, Kransdorf MJ, Bancroft LW, O'Connor MI. Malignant fatty tumors: Classification, clinical course, imaging appearance and treatment. Skeletal Radiol 2003;32:493-503.  Back to cited text no. 10
    

What is new? 1. Diffusion-weighted imaging, which showed signal intensity changes indicative of a highly cellular tumor in this case, is not routinely done in chondroid syringoma. 2. To our knowledge, this is the first case of chondroid syringoma mimicking low-grade liposarcoma reported in the literature


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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