Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Renal Collecting Duct Carcinoma

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4923-1

Synonyms

Definition

A highly malignant neoplasm arising from the principal cells of the renal collecting ducts of Bellini.

Clinical Features

  • Incidence

    Collecting duct carcinoma is a rare tumor accounting for 1–2% of renal neoplasms.

  • Age

    It occurs in a wide age range with the median age similar to the other renal carcinoma.

  • Sex

    There is a slight male predominance.

  • Site

    The tumor is centered on the renal medulla.

  • Treatment

    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.

  • Outcome

    Frequently the patients are symptomatic, with lymph node involvement and metastases to the lungs, liver, bone, adrenals, and brain at the time of diagnosis.

Macroscopy

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).

Microscopy

Histologically, they are high-grade carcinomas with ductal and papillary architecture (Fig. 1), stromal desmoplasia, and lymphocytic infiltration. Mitoses are numerous and often abnormal. Necrosis, sarcomatoid, or rhabdoid transformation is common, such as intratubular dysplasia in the adjacent renal parenchyma (Fleming and Lewi 1986).
Fig. 1

High-grade carcinomas with ductal and papillary architecture located in the medulla (a) with lymph node metastasis (b)

Immunophenotype

Collecting duct carcinomas express high molecular weight cytokeratins (CK19, 34βE12), CK7, vimentin, PAX8, INI1, and p63 (Kobayashi et al. 2008).

Molecular Features

Data are limited. Collecting duct carcinomas show several chromosomal aberrations in different chromosomes.

Differential Diagnosis

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

  1. Fleming, S., & Lewi, H. J. (1986). Collecting duct carcinoma of the kidney. Histopathology, 10, 1131–1141.CrossRefGoogle Scholar
  2. 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
  3. Kobayashi, N., Matsuzaki, O., Shirai, S., et al. (2008). Collecting duct carcinoma of the kidney: An immunohistochemical evaluation of the use of antibodies for differential diagnosis. Human Pathology, 39, 1350–1359.CrossRefGoogle Scholar
  4. Ohe, C., Smith, S. C., Sirohi, D., et al. (2018). Reappraisal of morphologic differences between renal medullary carcinoma, collecting duct carcinoma, and fumarate hydratase-deficient renal cell carcinoma. The American Journal of Surgical Pathology, 42, 279–292.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly