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Renal Cell Carcinoma, Unclassified with Medullary Phenotype and Synchronous Renal Clear Cell Carcinoma Present in a Patient with No Sickle Cell Trait/Disease: Diagnostic and Therapeutic Challenges.

Abstract Renal medullary carcinoma (RMC) is an aggressive high-grade renal cell carcinoma (RCC) associated almost exclusively with sickle cell trait or sickle cell disease. However, RCC with RMC features has rarely been reported in patients with no sickle cell trait or disease. Renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) is a newly-coined term used by an international panel of experts to describe renal cell carcinoma showing morphologic and immunohistochemical features of renal medullary carcinoma in patients without sickle cell trait/disease. So far, only one study in the English literature has described five such cases. Here, we report a case with unique clinical and pathological features in a 76-year-old male patient without sickle cell trait. The patient had a history of colon cancer with liver and lung metastases and was found to have a new renal mass in his right kidney during the follow up. A right nephrectomy was performed and showed two separate masses (tumor 1 and tumor 2). Tumor 1 had histologic features of RMC and the tumor cells were positive for CK7, Pax8, and OCT4 and showed loss of nuclear INI1 expression. Tumor 1 was diagnosed as RCCU-MP (6.3 cm, pT3aNx, WHO/ISUP nuclear grade 3). Tumor 2 showed features of clear cell type of RCC (0.6 cm, pT1aNx, WHO/ISUP grade 2) with intact nuclear INI1 expression. Three-months post-nephrectomy, the patient developed lung metastasis of RCCU-MP. To the best of our knowledge, this was the first documented case with synchronous RCCU-MP and clear cell RCC presenting in a patient without sickle cell trait. Careful histologic assessment with a panel of immunohistochemical biomarkers was helpful to render a correct diagnosis for early aggressive treatment.
PMID
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Authors

Mayor MeshTerms
Keywords

Kidney

diagnosis

medullary phenotype

prognosis

renal cell carcinoma

Journal Title anticancer research
Publication Year Start




PMID- 29848739
OWN - NLM
STAT- MEDLINE
DCOM- 20180611
LR  - 20180611
IS  - 1791-7530 (Electronic)
IS  - 0250-7005 (Linking)
VI  - 38
IP  - 6
DP  - 2018 Jun
TI  - Renal Cell Carcinoma, Unclassified with Medullary Phenotype and Synchronous Renal
      Clear Cell Carcinoma Present in a Patient with No Sickle Cell Trait/Disease:
      Diagnostic and Therapeutic Challenges.
PG  - 3757-3761
LID - 10.21873/anticanres.12657 [doi]
AB  - Renal medullary carcinoma (RMC) is an aggressive high-grade renal cell carcinoma 
      (RCC) associated almost exclusively with sickle cell trait or sickle cell
      disease. However, RCC with RMC features has rarely been reported in patients with
      no sickle cell trait or disease. Renal cell carcinoma unclassified with medullary
      phenotype (RCCU-MP) is a newly-coined term used by an international panel of
      experts to describe renal cell carcinoma showing morphologic and
      immunohistochemical features of renal medullary carcinoma in patients without
      sickle cell trait/disease. So far, only one study in the English literature has
      described five such cases. Here, we report a case with unique clinical and
      pathological features in a 76-year-old male patient without sickle cell trait.
      The patient had a history of colon cancer with liver and lung metastases and was 
      found to have a new renal mass in his right kidney during the follow up. A right 
      nephrectomy was performed and showed two separate masses (tumor 1 and tumor 2).
      Tumor 1 had histologic features of RMC and the tumor cells were positive for CK7,
      Pax8, and OCT4 and showed loss of nuclear INI1 expression. Tumor 1 was diagnosed 
      as RCCU-MP (6.3 cm, pT3aNx, WHO/ISUP nuclear grade 3). Tumor 2 showed features of
      clear cell type of RCC (0.6 cm, pT1aNx, WHO/ISUP grade 2) with intact nuclear
      INI1 expression. Three-months post-nephrectomy, the patient developed lung
      metastasis of RCCU-MP. To the best of our knowledge, this was the first
      documented case with synchronous RCCU-MP and clear cell RCC presenting in a
      patient without sickle cell trait. Careful histologic assessment with a panel of 
      immunohistochemical biomarkers was helpful to render a correct diagnosis for
      early aggressive treatment.
CI  - Copyright(c) 2018, International Institute of Anticancer Research (Dr. George J. 
      Delinasios), All rights reserved.
FAU - Lai, Jenny Z
AU  - Lai JZ
AD  - University College, Washington University in St. Louis, St. Louis, MO, U.S.A.
AD  - Division of Urologic Surgery, Department of Surgery, School of Medicine,
      Washington University in Saint Louis, St. Louis, MO, U.S.A.
AD  - Department of Pathology and Immunology, School of Medicine, Washington University
      in Saint Louis, St. Louis, MO, U.S.A.
FAU - Lai, H Henry
AU  - Lai HH
AD  - Division of Urologic Surgery, Department of Surgery, School of Medicine,
      Washington University in Saint Louis, St. Louis, MO, U.S.A.
FAU - Cao, Dengfeng
AU  - Cao D
AD  - Department of Pathology and Immunology, School of Medicine, Washington University
      in Saint Louis, St. Louis, MO, U.S.A. [email protected]
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - Greece
TA  - Anticancer Res
JT  - Anticancer research
JID - 8102988
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Aged
MH  - Biomarkers, Tumor/metabolism
MH  - Carcinoma, Renal Cell/metabolism/*pathology/surgery
MH  - Humans
MH  - Kidney/metabolism/*pathology/surgery
MH  - Kidney Medulla/metabolism/*pathology/surgery
MH  - Kidney Neoplasms/metabolism/*pathology/surgery
MH  - Lung Neoplasms/metabolism/secondary
MH  - Male
MH  - Nephrectomy/methods
MH  - Phenotype
MH  - Sickle Cell Trait/pathology
OTO - NOTNLM
OT  - Kidney
OT  - diagnosis
OT  - medullary phenotype
OT  - prognosis
OT  - renal cell carcinoma
EDAT- 2018/06/01 06:00
MHDA- 2018/06/12 06:00
CRDT- 2018/06/01 06:00
PHST- 2018/04/07 00:00 [received]
PHST- 2018/04/30 00:00 [revised]
PHST- 2018/05/02 00:00 [accepted]
PHST- 2018/06/01 06:00 [entrez]
PHST- 2018/06/01 06:00 [pubmed]
PHST- 2018/06/12 06:00 [medline]
AID - 38/6/3757 [pii]
AID - 10.21873/anticanres.12657 [doi]
PST - ppublish
SO  - Anticancer Res. 2018 Jun;38(6):3757-3761. doi: 10.21873/anticanres.12657.