Extramammary paget disease of the penoscrotal region.

Extramammary Paget disease is an uncommon slow growing intraepithelial malignant neoplasm. It can be classified into primary and secondary subtypes, with secondary subtype associated with underlying malignancy. Extramammary Paget disease of the penoscrotal region is extremely rare with very limited literature available describing its clinicopathologic characteristics. We present 6 cases of penoscrotal EMPD diagnosed at our academic medical center over a 20 year period. These included 5 cases of scrotal EMPD and 1 case of penile EMPD. The mean age at diagnosis was 68.6 yrs. (Range 61-78 years), One case of scrotal EMPD had history of renal cell carcinoma and prostatic adenocarcinoma, while one other case presented as recurrent EMPD with initial disease in the left groin. EMPD in the glans penis was associated with a history of urothelial carcinoma in the ureter with pagetoid spread. 3 cases had no progression of the disease till recent follow up, 2 were lost to follow up while 1 case rapidly deteriorated resulting in death. This case had bone metastatic and associated peritoneal carcinomatosis. Thus, Extramammary Paget disease of the penoscrotal area is extremely rare, can be primary or associated with visceral malignancies and usually tends to present at an older age. Peritoneal spread and distant metastasis are associated with rapid progression of the disease.

Risk of Lobular Neoplasia Upgrade with Synchronous Carcinoma.

BACKGROUND: Atypical lobular hyperplasia (ALH) and classic lobular carcinoma in situ encompass a spectrum of proliferative lesions known as lobular neoplasia (LN). When imaging-concordant and found in isolation on core needle biopsy (CNB), LN infrequently upgrades to carcinoma on surgical excision, and routine excision is not indicated. Upgrade rates in the setting of synchronous carcinoma are not well studied. PATIENTS AND METHODS: Patients with radiology-pathology concordant synchronous LN and separately biopsied ipsilateral (n = 35) or contralateral (n = 15) carcinoma who underwent excision between 2010 and 2021 were retrospectively identified. Frequency of upgrade, to either invasive or in situ carcinoma, was quantified, and factors associated with upgrade were assessed using Fisher's exact test. RESULTS: The median age was 55 (range 33-74) years. The upgrade rate of LN was 6% and not significantly different between ipsilateral (2.9%) and contralateral (13.3%) carcinoma (p = 0.15). All upgraded LN lesions were ALH on CNB and detected as non-mass enhancement on magnetic resonance imaging (MRI). No additional disease was demonstrated after excision at the site of the original LN CNB in 22.9% (8 out of 35) of ipsilateral and 13.3% (2 out of 15) of contralateral patients. Upgrade was not associated with family history, menopausal status, imaging modality used to detect LN, or extent of LN on CNB (p > 0.05). CONCLUSIONS: Our results demonstrate a low upgrade rate (6%) in our study cohort of LN with synchronous ipsilateral or contralateral carcinoma, which suggests that not all LN mandates excision with synchronous carcinoma. Larger, multi-institution studies are needed to validate these findings.