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- Presentation
Two Challenging Dermatopathology Cases: Combined Merkel-Squamous Carcinoma and Pediatric Nodular Hidradenoma
Description
This presentation reviewed two difficult dermatopathology cases. The first involved a 76-year-old man with a left calf squamous cell carcinoma that persisted and progressed despite radiation, immunotherapy, and cetuximab, eventually involving the left inguinal node and later a new right calf lesion. Biopsy of the right calf showed a tumor with both squamous and neuroendocrine features, including TTF1 positivity, CK20 and neuroendocrine marker expression, and high mutational burden without Merkel polyomavirus, leading to the diagnosis of a combined Merkel cell and squamous cell carcinoma rather than metastatic lung disease or pure SCC. The case emphasized careful clinicopathologic correlation, nodal staging, viral testing, and serial imaging, as well as the aggressive behavior of combined tumors. The second case described a four-year-old boy with a rapidly enlarging abdominal lesion initially thought to be an insect bite. Histology showed a nodular hidradenoma with concerning features including increased mitoses, deep infiltrative growth, pleomorphism, and vascular invasion, leading to a diagnosis of atypical nodular hidradenoma with a focus of hidradenocarcinoma. The speaker reviewed the overlapping terminology and diagnostic criteria for hidradenoma and hidradenocarcinoma, noted the rarity of pediatric cases, and stressed that malignancy should be called only after considering the full pattern of findings. Both cases highlighted the importance of expert consultation and multidisciplinary management in rare, challenging skin tumors.
View moreConclusions
- The first case suggests that combined Merkel cell and squamous cell carcinoma is a rare but highly aggressive cutaneous tumor that can be diagnostically confusing, especially when TTF1 is positive and the patient already has metastatic squamous cell carcinoma elsewhere.
- Because combined Merkel cell/squamous tumors may arise from UV-related pathways rather than true pulmonary or classic dermal neuroendocrine origins, diagnosis must rely on the full clinicopathologic context, not any single marker.
- For suspected combined Merkel cell/squamous carcinoma, aggressive management with nodal evaluation, polyomavirus testing, and serial surveillance imaging is warranted.
- The second case shows that an apparently benign, well-circumscribed adnexal tumor in a child can still behave malignantly when deep infiltration, vascular invasion, higher mitotic activity, and pleomorphism are present.
- Atypical nodular hidradenoma appears to be a borderline entity that overlaps with hidradenocarcinoma, and classification should be based on the overall pattern of aggressive features rather than one isolated finding such as intravascular invasion.
- Hydradenocarcinoma is extremely rare but can recur locally and metastasize, so complete excision, nodal assessment, and close follow-up are important despite limited formal guidelines.
- The presentation emphasizes that rare skin tumors often require cautious expert review because histopathologic discordance is common and pediatric cases deserve especially careful interpretation and management.
- Cutaneous squamous and neuroendocrine carcinoma: genetically and immunohistochemically different from Merkel cell carcinoma.#10.1038/modpathol.2015.60
- TTF-1 and PAX5 Are Frequently Expressed in Combined Merkel Cell Carcinoma.#10.1097/dad.0000000000000464
- A 2023 Journal of Cutaneous Pathology article about combined Merkel cell carcinoma and cutaneous squamous cell carcinoma with lymph node metastases.
- Nodular hidradenoma
- Hidradenocarcinoma
- Clear Cell Nodular Hidradenoma Involving the Lymphatic System: A Tumor of Uncertain Malignant Potential or a Novel Example of 'Metastasizing' Benign Tumor?#10.3410/f.717967985.793467756
- Malignant Transformation of Retroauricular Hidradenoma in Hidradenocarcinoma in a Nine-Year-Old Patient: A Case Report.#10.7759/cureus.37160
- Case 1 selected references