| Abstract|| |
A 25-year-old woman was seen for a painless subcutaneous mass of 2 years duration. On excisional biopsy, a collapsed cystic structure lined by stratified, ciliated, columnar epithelium was noted. These linning cells did not produce mucin. Immunohistochemical staining for progesterone receptor, estrogen receptor and epithelial membrane antigen was positive, whereas it was negative for carcinoembryonic antigen. Findings were consistent with cutaneous ciliated cyst (CCC). CCCs are rare, predominantly occurring on the lower extremities of young women. Most of them have been regarded as Mullerian remnants. A case of a CCC in the subcutaneous area is reported.
Keywords: Cutaneous ciliated cyst, immunohistochemistry, subcutaneous area
|How to cite this article:|
Gelincik I. Cutaneous ciliated cyst in the subcutaneous area. Indian J Pathol Microbiol 2011;54:150-1
| Introduction|| |
Cutaneous ciliated cyst (CCC) is an unusual cystic lesion of the skin, which preferentially affects the lower limbs of young females.  Cutaneous cysts lined by ciliated epithelium are very rare.  Only 40 well-documented cases of cutaneous ciliated cysts have been reported in the published work. CCC typically presents as a single unilocular or multilocular subcutaneous cyst lined by epithelium, histologically reminiscent of Fallopian tube More Details mucosa, on the lower extremity or buttock of young girls, shortly after puberty. 
| Case Report|| |
A 25-year-old woman presented with a 2-year history of a mass in the subcutaneous area. According to the patient, the mass was painless, but had gradually increased in size. Laboratory investigation results including complete blood cell count and blood chemistry were normal. After surgical removal, examination of the cyst wall resulted in the escape of clear serous fluid from the cystic cavity. Histopathologic examination revealed a uni-loculated cyst located in the deep dermis. Scanning magnification revealed a collapsed cystic structure lined by stratified, ciliated, columnar epithelium, resembling the fimbriated portion of the fallopian tube [Figure 1]. Immunohistochemical analysis revealed positive staining for epithelial membrane antigen and cytoplasmic staining for keratin, which proved the epithelial origin of the tumor. The cyst did not stain for carcinoembryonic antigen and S-100. No evidence of muscular differentiation was observed, as evidenced by the negative staining of desmin and actin. There was a strong nuclear staining for progesterone receptor and estrogen receptor [Figure 2] in many of the epithelial cells. Inflammatory infiltrate was absent in and around the cyst.
|Figure 1: Histopathologic features of a cystic structure lined by stratified, ciliated, columnar epithelium in the dermis [H and E, (a) ×100; (b) ×100]|
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|Figure 2: Cutaneous ciliated cyst. (a) Epithelial cells are positive for progesterone receptors (IHC, ×400); (b) epithelial cells are positive for estrogen receptors (IHC, ×400)|
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| Discussion|| |
Since the original report of CCC by Hess  in 1890, there have been only 40 reported cases of CCCs. ,, On electron microscopy, the ciliary shaft contains an array of nine peripheral microtubule pairs arranged in a circle around two single microtubules. , This finding is identical to that of normal human ciliated cells found in the epithelium of the airway, fallopian tube, middle ear and in the ependymal lining of the brain and spinal cord. Also, similar immunohistochemical staining patterns for dynein, which functions as microtubule-based molecular motors, are seen in both cutaneous ciliated cysts and fallopian tube epithelium.  Negative carcinoembryonic antigen and S-100 with positive staining for estrogen receptor and/or progesterone receptor verified the CCCs' nature to be of fallopian tube epithelium.  In the present case, immunohistochemically, there was a strong nuclear staining for progesterone receptor and estrogen receptor in many of the epithelial cells. The cyst did not stain for carcinoembryonic antigen and S-100.
A CCC is a benign cystic lesion that is often a palpable subcutaneous mass without any symptoms. In this case, immunohistochemical analysis for the presence of progesterone receptors and estrogen receptor was positive, which supports the theory of heteropia of the ciliated epithelium from the Mullerian epithelium because the Mullerian duct is the precursor of the fallopian tubes and the body and cervix of the uterus, which are known to stain for progesterone receptors and estrogen receptors. 
It is believed that cutaneous ciliated cysts are a distinct entity occurring in females, probably arising from hormonal activation of a Mullerian rest.
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Bolge Egitim ve Arastirma Hastanesi, Patoloji Klinigi Cat Yolu cad, Yildizkent-Erzurum
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]