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LETTER TO EDITOR  
Year : 2019  |  Volume : 62  |  Issue : 1  |  Page : 173-174
Primary cutaneous mucinous carcinoma of the chin: Report of a case


1 Department of Plastic and Recostructive Surgery, Ospedale Universitario Santa Maria Della Misericordia, Udine, Italy
2 Department of Pathology, Ospedale Universitario Santa Maria Della Misericordia, Udine, Italy

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Date of Web Publication31-Jan-2019
 

How to cite this article:
Fin A, D'Alì L, Mura S, Cordaro ER, De Biasio F, Mariuzzi L, Parodi PC. Primary cutaneous mucinous carcinoma of the chin: Report of a case. Indian J Pathol Microbiol 2019;62:173-4

How to cite this URL:
Fin A, D'Alì L, Mura S, Cordaro ER, De Biasio F, Mariuzzi L, Parodi PC. Primary cutaneous mucinous carcinoma of the chin: Report of a case. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Apr 25];62:173-4. Available from: http://www.ijpmonline.org/text.asp?2019/62/1/173/251235




Editor,

Primary cutaneous mucinous carcinoma (PCMC) is a rare skin cancer arising from sweat glands. According to available data, PCMC is extremely rare – fewer than 200 cases of PCMC have been reported in the literature. Although it is associated with good prognosis and low metastatic rate (10%), PCMC has a high rate of local recurrence (30%), and differential diagnosis can be problematic. Surgeons and pathologists should, therefore, be aware of its clinical and pathological features, which may easily be confused with those displayed by other benign or malignant lesions such as cystic lesions, sarcoma, and squamous or basal cell carcinoma.[1] This means that the actual incidence of PCMC may be higher, and it is particularly important to exclude cutaneous metastasis from distant adenocarcinomas.

The typical presentation site of PCMC is the head. However, we present a very unusual case of PCMC of the chin [2] – a 62-year-old white male referred to our center for a prominent asymptomatic and slowly growing mass that had appeared on the chin 20 years before [Figure 1]. Examination revealed a mobile, firm, pink, telangiectatic, translucent nodule measuring 5 cm × 3 cm × 4.5 cm. Local lymph node examination was negative, and with the exception of a personal history of alcohol abuse and heavy smoking, the anamnesis was unremarkable. Dermatological examination of a biopsy specimen showed numerous islands of epithelial tumor cells “floating” in lakes of basophilic mucin. These cells presented few mitotic figures, round-to-cuboidal nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Thin fibrous septa separated the large pools of mucin, forming a “honeycomb” pattern. Immunohistochemical profiling of tumor cells showed positivity for cytokeratin-7 (CK7), estrogen, and progesterone receptors; negativity for cytokeratin-20 (CK20), homeobox transcription factor (CDX2), and mucus-associated peptides of the thyroid transcription factor-1 and P63; and mucin positivity for alcian blue at pH 2.5 [Figure 2]. These features were characteristics of PCMC.[3] p63 expression has been advocated as a valuable tool in distinguishing between primary cutaneous adnexal carcinoma and primary/metastatic visceral adenocarcinoma, the latter being usually negative for p63. As this case demonstrates, a panel of markers along with a complete clinical workup is essential to establish the diagnosis.[4] PCMC can be distinguished from gastrointestinal tumors based on mucin histochemistry and immunohistochemistry: PCMC is CK7+ and CK20−, in contrast to gastrointestinal cancers, which show reversed positivity. Although more men than women are affected, a full clinical workup is essential to rule out cancers of a mammary or ovarian origin.[5] The patient underwent radical excision of the tumor as the first-choice treatment for PCMC is surgery. Preoperative assessment – which included routine blood tests; electrocardiography; thoracic X-ray; and facial, abdominal, and pelvic computed tomography (CT) scans to exclude local invasion or distant metastases – showed no relevant findings. CT scan of the facial bones showed no signs of mandibular infiltration. Given the high recurrence rate, authors recommend considering >1 cm of healthy margins as safe although a systematic review and meta-analysis of 159 cases performed by Kamalpour et al.[6] suggest that outcome after surgical excision depends on age, race, size, location of the tumor, and follow-up rather than on surgical margins. Although available reports indicate that margin size seems not to significantly influence the relapse rate, long-term follow-up data are scarce, and the excision was performed taking care to maintain a visible healthy margin of 2 cm, and reconstruction was delayed until histology confirmed clear >1 cm margins (2 cm from the superior margin, 1 cm from the inferior, 1.2 cm from the right, and 1.5 cm from the left).
Figure 1: Clinical presentation of primary cutaneous mucinous carcinoma after the biopsy specimen was taken. Note the telangiectatic, translucent aspect of the growth. Informed consent for these images was obtained from the patient

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Figure 2: (a) H and E, ×2 islands of epithelial tumor cells “floating” in lakes of basophilic mucin and forming a “honeycomb” pattern. (b) CK7 2X v2: Tumor cells showing positivity for cytokeratin-7, cytokeratin-20, progesterone receptor, and estrogen receptor

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The residual defect, measuring 7.5 cm × 6.7 cm, was repaired with a bilateral advancement flap according to the Burow technique, which produced good esthetic outcome and patient satisfaction. Clinical examination of the patient was performed every 3 months, and no relapse occurred during 20 months of follow-up.

In addition to the need for further and more recent follow-up data regarding previously reported cases of PCMC, this case highlights a requirement for PCMC to be considered in differential diagnosis of an indolent, asymptomatic, cutaneous, or subcutaneous neoformation of the neck, face, scalp, or eyelids. Total body scans are recommended to exclude primitive carcinoma or distant PCMC metastases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Scholz IM, Hartschuh W. Primary mucinous eccrine carcinoma of the skin – A rare clinical tumor with many differential diagnoses. J Dtsch Dermatol Ges 2010;8:446-8.  Back to cited text no. 1
    
2.
Breiting L, Christensen L, Dahlstrøm K, Breiting V, Winther JF. Primary mucinous carcinoma of the skin: A population-based study. Int J Dermatol 2008;47:242-5.  Back to cited text no. 2
    
3.
Wright JD, Font RL. Mucinous sweat gland adenocarcinoma of eyelid: A clinicopathologic study of 21 cases with histochemical and electron microscopic observations. Cancer 1979;44:1757-68.  Back to cited text no. 3
    
4.
Kanitakis J, Chouvet B. The usefulness of P63 detection for differentiating primary from metastatic skin adenocarcinomas. J Cutan Pathol 2008;35:692-3.  Back to cited text no. 4
    
5.
Wick MR. Primary lesions that may imitate metastatic tumors histologically: A selective review. Semin Diagn Pathol 2018;35:123-42.  Back to cited text no. 5
    
6.
Kamalpour L, Brindise RT, Nodzenski M, Bach DQ, Veledar E, Alam M, et al. Primary cutaneous mucinous carcinoma: A systematic review and meta-analysis of outcomes after surgery. JAMA Dermatol 2014;150:380-4.  Back to cited text no. 6
    

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Correspondence Address:
Alessandra Fin
Clinic Department of Plastic and Reconstructive Surgery, Ospedale Univeristario Santa Maria Della Misericordia, Piazzale Santa Maria Della Misericordia 15, 33100 Udine
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_136_18

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