Primary Cutaneous Mucinous Neoplasm

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Primary Cutaneous Mucinous Carcinoma (PCMC) is an exceptionally rare malignant tumor originating from the skin's sweat glands. First identified by Lennox et al. in 1952, PCMC has seen approximately 215 documented cases in medical literature since then.

Epidemiology and Demographics

PCMC predominantly affects individuals between the ages of 50 and 70, with a slight male predominance. The tumor most commonly arises in the head and neck region, particularly the eyelids, accounting for about 41% of cases. Other affected areas include the scalp (17%), face (14%), axilla (9%), chest and abdomen (7%), vulva (4%), neck (2%), extremities (2%), canthus (2%), groin (1%), and ear (1%).

Geographically, PCMC has a higher prevalence among white individuals (77.2%) compared to Asians (12.7%) and African Americans (10.1%). However, a population-based study analyzing data from 1972 to 2013 identified 411 cases of PCMC, suggesting that the incidence may be higher than previously recognized.

Clinical Presentation

Clinically, PCMC presents as a slow-growing, painless nodule that can vary in color from translucent to reddish or gray-blue. Lesions typically range from 0.7 to 8 cm in size and may be present for many years before diagnosis. Due to its indolent nature and nonspecific appearance, PCMC is often mistaken for benign conditions such as cysts or other skin tumors.

Diagnosis and Treatment

In this case, ultrasound imaging revealed a well-circumscribed, hypoechoic mass located in the dermis and subcutaneous tissue. The lesion exhibited posterior acoustic enhancement, a feature often associated with fluid-filled structures or lesions containing mucin. No significant internal vascularity was noted on Doppler imaging, suggesting a lack of prominent blood flow within the tumor.

It's important to note that while this case provides valuable information, the ultrasound appearance of PCMC can vary. Given the limited data, further studies are necessary to establish a comprehensive understanding of the sonographic features of PCMC. Clinicians should consider PCMC in the differential diagnosis when encountering well-defined, hypoechoic dermal or subcutaneous masses with posterior acoustic enhancement, especially in patients presenting with slow-growing skin nodules.



Histologically, PCMC is characterized by nests of neoplastic epithelial cells floating in mucinous lakes. Immunohistochemical studies are essential to distinguish PCMC from metastatic mucinous carcinomas originating from other sites, such as the breast or gastrointestinal tract. Markers like CK7, CK20, CDX2, TTF1, D2-40, and p63 are utilized in this differentiation process.

The standard treatment for PCMC is surgical excision with clear margins. Despite its low metastatic potential, PCMC has a high local recurrence rate, reported between 26% and 40%. Therefore, regular follow-up is crucial to monitor for recurrence.

Conclusion

Primary Cutaneous Mucinous Carcinoma is a rare and often under-recognized skin malignancy. Due to its indolent presentation and potential for misdiagnosis, heightened awareness among clinicians is essential for early detection and appropriate management. Further research is needed to better understand its pathogenesis, optimal treatment strategies, and long-term outcomes.

Location: Nigeria

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