1. SPLIT, Croatia AMR Slide Seminar Case # 29 Kidney tumor
Ivan Damjanov, MD, PhD
Department of Pathology
The University of Kansas School of Medicine, Kansas City, Kansas

2. Case # 29 Clinical History
26-year-old African American woman complaining of lower abdominal and lumbar pain
CT-scan –left kidney tumor and hepatic metastases
Lab findings- mild anemia, LDH ↑and sickle cell trait
Negative tumor markers (AFP, hCG, CEA)

3. Case # 38 Pathology
Macro: Renal tumor measuring 12x 7 x 6.5 cm with 20% necrosis. Tumor extending into the surrounding perinephric fat tissue and into the peri-adrenal fat tissue and renal vein
Enlarged regional lymph nodes, positive for malignancy
Stage: pT3a
Micro: Metastasis in the perirenal hilar lymph nodes
Stage: p3a,N1,M1

4. Reticular the predominant pattern

5. Reticular and solid

6. Reticular with prominent mucoid stroma

7. Cribriform –sieve like and solid

8. Yolk sac like,loose stroma, inflammatory cells

9. Loosely structured permeated with blood

10. Solid, cord nests and cystic

11. Solid,invasive cords and ,nests

12. Cribriform and clear cell

13. Clear cell reticular and mucinous

14. Infiltrating with desmoplastic inflammatory reaction

17. Case # 29 Immunohistochemistry
Positive: Pancytokeratin, CK7, vimentin, PAX8, OCT4
Negative: SMARCB1(INI1)

21. Diagnosis: Renal medullary carcinoma

22. Renal medullary carcinoma-quick review
Rare, highly aggressive carcinoma of the kidney
First described by Davis, Mostofi and Sesterhenn (AJSP,1995) as “seventh sickle cell nephropathy”
Most patients are positive for sickle cell trait, but there are well documented exceptions
Median survival 4-5 months, essentially all die by 3 years

23. Renal medullary carcinoma-microscopic
Variety of patterns of which the most common are the following
( see Ohe C et al,AJSP 2018;42:279— multicenter study, AMR members participated: Abbas Agaimy and Ondra Hes)
Solid, cord, nests (97%)
Cribriform, sieve like (88%)
Reticular (yolk sac tumor like) 85% (associated usually with cribriform)
Stromal myxoid change (94%)—on gross examination appears mucoid!!
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Infiltrating glandular (55%)
Tubular/papillary ( 35%)
Sarcomatoid change (15%),rhabdoid cells,desmoplasia

24. Renal medullary carcinoma- immunohistochemistry
Confirm that it is renal and not a metastasis or germ cell tumor (e.g., yolk sac carcinoma)
Watch out: RMC is often positive for OCT4!
Exclude other high grade distal nephron-related adenocarcinomas (collecting duct carcinoma and fumarate hydratase-deficient RCC)
Confirm the diagnosis with antibodies to SMRCB1(INI1)

25. Renal medullary carcinoma- unresolved questions-take home message(if any!?)
Clinical data are important—typically: aggressive carcinoma in a young person of African origin with sickle cell trait.
SMARCB1 (INI1) loss by immunohistochemistry- diagnostic (Note: Rhabdoid kidney tumors also lack SMARCB1)
Molecular questions: SMARCB1 rearrangement/fusion vs. loss?
No consensus among uropathologists if sickle cell trait is an obligate criterion for diagnosis RMC or is it enough demonstrate a
“SMARCB1-opathy”.
Lets watch out for the proposed groups of tumors called
Renal cell carcinoma, unclassified, with medullary phenotype (Sirohi et al.Hum Pathol 2017; 67:134)

26. The End

27. SPLIT,Croatia AMR Slide Seminar Case # 76 Ovarian tumor
Ivan Damjanov, MD, PhD
Department of Pathology
The University of Kansas School of Medicine, Kansas City, Kansas

28. Case # 76 Clinical History
60-year-old woman complaining of lower abdominal and pelvic pain
Past history: 30 years ago had “her uterus and tubes removed”
Left ovarian mass measuring 15 cm was found partially attached to the loops of the large intestine. Several tumor nodules on the mesentery and several pelvic lymph nodes contained tumor on frozen section. Right ovary contained an adenofibroma. Radical removal of all the neoplastic tissue was attempted.
She received chemotherapy. Two years after surgery she was found to have liver metastases. Lost to follow up, presumably died of spreading tumor.

29. Case # 38 Macroscopic and Intraoperative Pathology
Macro: Tumor received in several parts .Tissue soft friable and partially necrotic.
Partial colectomy with tumor attached to the intestine.
Partial omentectomy and lymph node dissection with apparent tumor.
Frozen sections: Malignant tumor, favor adenocarcinoma.
Metastases in the submitted lymph nodes and omentum.