Renal Collecting Duct Carcinoma
A highly malignant neoplasm arising from the principal cells of the renal collecting ducts of Bellini.
Collecting duct carcinoma is a rare tumor accounting for 1–2% of renal neoplasms.
It occurs in a wide age range with the median age similar to the other renal carcinoma.
There is a slight male predominance.
The tumor is centered on the renal medulla.
Radical nephrectomy is the standard of care, often with local lymphadenectomy. Since metastases are frequently present at the moment of the diagnosis, the surgical excision is followed by chemotherapy.
Frequently the patients are symptomatic, with lymph node involvement and metastases to the lungs, liver, bone, adrenals, and brain at the time of diagnosis.
They are usually large and infiltrative, whitish, and firm tumors grossly located in the medulla or central parts of the kidney (Fleming and Lewi 1986).
Collecting duct carcinomas express high molecular weight cytokeratins (CK19, 34βE12), CK7, vimentin, PAX8, INI1, and p63 (Kobayashi et al. 2008).
Data are limited. Collecting duct carcinomas show several chromosomal aberrations in different chromosomes.
The main differential diagnoses are high-grade papillary renal cell carcinoma, invasive urothelial carcinoma, adenocarcinoma of the renal pelvis, and renal medullary carcinoma. The latter neoplasm occurs in younger patients with sickle cell trait and often the neoplastic cells express Oct3/4 which is negative in collecting duct carcinoma. In the differential diagnosis with urothelial carcinoma, the recognition of the carcinoma in situ of the urothelium is extremely useful because PAX8 may be expressed up to 20% of urothelial carcinomas and GATA3 may be negative in high-grade urothelial carcinomas (Gupta et al. 2012; Ohe et al. 2018).
References and Further Reading
- Gupta, R., Billis, A., Shah, R. B., et al. (2012). Carcinoma of the collecting ducts of Bellini and renal medullary carcinoma: Clinicopathologic analysis of 52 cases of rare aggressive subtypes of renal cell carcinoma with a focus on their interrelationship. The American Journal of Surgical Pathology, 36, 1265–1278.CrossRefGoogle Scholar