Case for diagnosis^*

Caso para diagnóstico

Abstracts

The chondroid syringoma is a rare benign tumor, also called mixed cutaneous tumor by the presence of epithelial and mesenchymal components, consisting of sweat elements in cartilaginous, collagenous, myxoid or osseous stroma, among others. It mainly affects middle-aged men and is characterized by asymptomatic and slowgrowing, dermal or subcutaneous nodules. The most common locations are the head and neck. It is rare on the extremities. There are reports of malignant variants predominantly in women, located on the extremities. We report a case of a female patient with a lesion on the toe, with excellent outcome after surgical treatment.

Cartilage; Extremities; Syringoma

O siringoma condroide é um tumor benigno, raro, também chamado de tumor misto cutâneo pela presença de componentes epiteliais e mesenquimais, que consistem em elementos sudoríparos em estroma cartilaginoso, colagênico, mixoide ou ósseo, entre outros. Acomete, principalmente, homens de meia-idade e caracteriza-se por nódulos subcutâneos ou dérmicos, assintomáticos e com crescimento lento. As localizações preferenciais são a cabeça e o pescoço, sendo raro nas extremidades. Existem relatos de variantes malignas, com predomínio em mulheres, localizadas nas extremidades. Relatamos um caso em paciente do sexo feminino, com lesão em extremidade e excelente resultado após o tratamento cirúrgico.

Cartilagem; Extremidades; Siringoma

CASE REPORT

A 68-year-old white female patient complained of an asymptomatic nodular lesion on the second left toe, with onset 7 years before and progressive increase in size since then. At physical examination a voluminous nodular lesion was observed, with smooth erythematous surface, telangiectasias and 4cm in diameter (Figure 1). There were no palpable swollen inguinal lymph nodes.

FIGURE 1
voluminous nodular lesion with a smooth erythematous surface with telangiectasias

A lesion biopsy was performed and the histopathological examination revealed cuboidal cell erytheagglomerates with basophilic round nucleus and eosinophilic cytoplasm adjacent to cartilaginous tissue (Figure 2).

FIGURE 2
cuboidal cells agglomerates with basophilic round nucleus and eosinophilic cytoplasm (Fig. 2, full arrow) adjacent to cartilaginous tissue (Fig. 2, empty arrow). HE 200x

A radiography of the left foot revealed increase in soft tissue without bone involvement.

DISCUSSION

In face of the clinical picture the following diagnostic hypotheses were considered: schwannoma, neurofibroma, nodular tenosynovitis, dermatofibroma and dermatofibrosarcoma protuberans. The histopathological examination indicated it was a chondroid syringoma (CS). The lesion was completely excised, with excellent esthetic and functional results (Figure 3).

FIGURE 3
images before and after the lesion was excised

The CS is a benign tumor also called mixed cutaneous tumor due to the presence of epithelial and mesenchymal components.^1-3 In 1961, Hirsch and Helwig were the first to use the term CS to describe the presence of sweat gland elements in cartilaginous stroma.^1-5 It is a rare tumor, with incidence between 0.01 and 0.1%.³ It affects mainly middle-aged men and is characterized by dermal or subcutaneous nodules, asymptomatic and of slow growth. It is typically located on the head and neck, only rarely on the extremities.^1-7 There have been reports of malignant variants, predominantly in women and located on the extremities.^1,3,5,8 Although our patient was female, with a lesion on the extremity, the histopathological study of the surgical biopsy confirmed its benign nature.

The neoplasm is located in the deep dermis and/or subcutaneous, forming a well defined and symmetrical tumor mass, delimited by fibrous septa. No pleomorphism, atypical mitoses and necrosis are found. It is currently believed that it might have an eccrine or apocrine origin, with the latter being the most common. The epithelial component has apocrine characteristics when it presents tubular and/or ductal structures with cuboidal cells internally and myoepithelial cells externally. In some cases, apocrine decapitation, follicular and sebaceous differentiation foci can be noted. A tumor is considered as having eccrine characteristics when epithelial stroma is composed of diminutive glands, with a single layer of cells, or of small islands of cuboidal cells dispersed in a myxoid or chondroid stroma. However, the differentiation between eccrine and apocrine is not always simple, either by histopathology or by immunohistochemistry. Even by electronic microscopy this differentiation may be unclear. The mesenchymal component is composed of myxoid or densely collagenized areas. The chondroid areas are observed in 59% of the cases. They may also present calcification foci, trabeculae and bone marrow.^1,9

The treatment of choice of CS is surgical.^1-9 As it is a lobulated type of tumor, it is recommended to include normal tissue margins to ensure that the entire tumor is removed.³

REFERENCES

• ¹
  Santos OLR, Silva CM, Souto LA, Harris OMO, Azulay DR, Pereira Júnior AC. Chondroid syringoma: analysis of four cases. An Bras Dermatol. 1992;67:145-50.
• ²
  Yavuzer R, Basterzi Y, Sari A, Bir F, Sezer C. Chondroid syringoma: a diagnosis more frequent than expected. Dermatol Surg. 2003;29:179-81.
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  Sivamani R, Wadhera A, Craig E. Chondroid syringoma: case report and review of the literature. Dermatol Online J. 2006;12:8.
• ⁴
  Beals TC, Nickisch F, Teman C, Florell SR, Layfield LJ. Chondroid syringoma: case report of a highly unusual foot tumor. Foot Ankle Int. 2010;31:333-5.
• ⁵
  Borman H, Deniz LM, Ertas NM, Arikan U, Colak A. Chondroid syringoma of the hand. Scand J Plast Reconstr Surg Hand Surg. 2009:43:291-3.
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  Nemoto K, Kato N, Arino H. Chondroid syringoma of the hand. Scand J Plast Reconstr Surg Hand Surg. 2002;36:379-81.
• ⁷
  MacKIE RM. Tumours of the skin appendages. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Rook/Wilkison/Ebling textbook of dermatology. 8th ed. New York: Blackwell Science; 2010. p.1709-1710.
• ⁸
  Shashikala P, Chandrashekhar HR, Sharma S, Suresh KK. Malignant chondroid syringoma. Indian J Dermatol Venereol Leprol. 2004;70:175-6.
• ⁹
  Salama ME, Azam M, Ma CK, Ormsby A, Zarbo RJ, Amin MB, al. Chondroid syringoma: cytokeratin 20 immunolocalization of Merkel cells and reappraisal of apocrine folliculo-sebaceous differentiation. Arch Pathol Lab Med. 2004;128:986-90.

• *
  Study carried out at the Dermatology Service of the Teaching Hospital Gaffrée e Guinle, Federal University of the State of Rio de Janeiro (Universidade Federal do Estado do Rio de Janeiro - HUGG-UNIRIO) - Rio de Janeiro (RJ), Brazil.
• Financial funding: None

Publication Dates

• Publication in this collection
  Nov-Dec 2013

History

• Received
  05 Feb 2013
• Accepted
  13 Mar 2013