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Slide Seminar- IV Pathology of Renal Tumors. Satish Tickoo Associate Prof. of Pathology Chief, Urologic Pathology Weill Medical College of Cornell University New York, NY. Slide Seminar- IV Pathology of Renal Tumors. Case 1: 55 years female with 3.5 cms well circumscribed left renal mass
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Slide Seminar- IVPathology of Renal Tumors Satish Tickoo Associate Prof. of Pathology Chief, Urologic Pathology Weill Medical College of Cornell University New York, NY
Slide Seminar- IVPathology of Renal Tumors • Case 1: 55 years female with 3.5 cms well circumscribed left renal mass • Case 2: 42 year male with 4,5 cms partly cystic left renal mass • Case 3: 38 year female with a mediastinal metastatic carcinoma & right renal tumor
Slide Seminar- IV : Pathology of Renal Tumors - Case 1 Case 1 • A 55 year old female with an “incidental” left renal mass. • The 3.5 cm well-circumscribed solid mass had a homogeneous tan-white cut-surface. • Tumor was extruding into the renal hilar fat without grossly invading it.
Slide Seminar- IV : Pathology of Renal Tumors - Case 1 Case 1 ? Diagnosis
Slide Seminar- IV : Pathology of Renal Tumors - Case 1 Case 1 • The sections from the tumor show a combination of epithelial and spindle cell components both of which are of relatively low-grade cytology. The epithelial component is in the form of small tubules with round profiles, elongated, branching tubules many appearing straight and with slit-like lumina, and solid compressed cord-like structures. The epithelial component merges imperceptibly with a low-grade spindle cell component. Mitotic activity is at the most very scant. No papillary areas are identified. The stroma is focally, myxoid, and shows occasional foamy macrophages. • The combination of these morphologic features is characteristic of a recently described entity:
Slide Seminar- IV : Pathology of Renal Tumors - Case 1 Case 1 • Mucinous Tubular and Spindle Cell Carcinoma (Previously Also Called Low-grade Biphasic RCC of Possible Collecting Duct/ Loop of Henle Origin)
Slide Seminar- IV : Pathology of Renal Tumors - Case 1 Case 1 • It is a tumor mostly described in females, at an average age of 53 years (range 17-78 years). Relatively fewer cases have occurred in males (M to F ratio, 1:4). The tumor is often centered in the renal medulla, and is typically well circumscribed, with a solid homogeneous tan-white cut surface. Hemorrhage and necrosis are not present, and usually no cysts are identified. • Although, the morphologic features are highly characteristic, in the past such tumors were included among RCC, unclassified. Superficial resemblance to type 1 (solid) papillary RCC and the presence of prominence of spindle cell areas in some has resulted in some cases being considered as sarcomatoid (papillary) RCC. • On immunohistochemical staining, the tumors are positive for CK7, AMACR, EMA, ulex europaeus, and peanut agglutinin, with fewer staining for high molecular weight cytokeratin and CD10. • The prognosis seems to be good, and regional lymph node metastasis has been described in only one case to date
Slide Seminar- IV : Pathology of Renal Tumors - Case 1 End of Cases 1
Case 2 Slide Seminar- IV : Pathology of Renal Tumors - Case 2 • A 4.5 cm, hemorrhagic, yellow-brown, partly cystic left renal mass in a 42 year old male, who had been on dialysis for the last 7 years.
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 CK7 p504s
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 Case 2 ? Diagnosis
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 Case 2 • The figures show the tumor with tubular, micro- and macro-cystic, solid, and an unusual cribriform architecture. • The cells are predominantly eosinophilic, with grade 3 nuclei. • Focal papillary architecture, and even clear cell RCC-like areas are also present. The cribriform areas show both intracytoplasmic and intercellular round to irregular small spaces. • Intratumoral oxalate crystals are also identified. • Background renal parenchyma shows an end-stage kidney with acquired cystic disease of the kidney (ACDK) (figure 6).
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 Case 2 • This is a very unusual tumor that we have observed only in the setting of ACDK. • It has been variably considered as a papillary RCC, clear cell RCC, or “solid RCC” by various authors in the past. However, the combination of the micro-cribriform areas, high-grade eosinophilic cytology, and abundant intratumoral oxalate crystals distinguishes them from other renal tumors, in spite of areas of papillary architecture and focal clear cell features. • Unlike papillary RCC, we have found these tumors to be usually completely negative for CK7. Also, unlike papillary RCC, in the small number of cases that we tested by FISH, these tumors have shown no trisomies for chromosomes 7 and 17, but often monosomies of these chromosomes.
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 Case 2 • These tumors are the commonest subtype of RCC arising in ACDK, although, in such kidneys other tumor types including papillary RCC, clear cell RCC, chromophobe RCC and papillary RCC with completely clear cell cytology are also common. • While the current designation for this tumor would be RCC, unclassified, we have tentatively designated them as: • ACDK-SPECIFIC RCC
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 Case 2 • Although, RCC arising in the background of ACDK has generally been considered to be less aggressive, we have seen metastases in 3 of our 60 cases. One of these 3 had sarcomatoid features, and that patient died of widespread metastases. Interestingly, all 3 cases with metastases were ACDK-specific RCC.
Slide Seminar- IV : Pathology of Renal Tumors - Case 2 End of Case 2
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 Case 3 • A 38 year old female, who presented with a mediastinal metastatic carcinoma, was found to have a 5.0 cm right renal tumor on evaluation for the metastasis.
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 AE1/AE3 Cam 5.2 HMWCK
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 Vimentin
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 Case 3 ? Diagnosis
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 Case 3 • This tumor shows predominantly clear cell cytology. Most areas are composed of solid sheets, nests and alveoli, separated by thin intricate fibrovascular septa. These features strongly suggest the diagnosis of a clear cell RCC. However, there are clear-cut areas of papillary architecture. Immunohistochemical features are also unusual for either a clear cell or a papillary RCC (both are usually vimentin, as well as, keratin positive). • Again, going by the definition of RCC, unclassified, when you see a combination of patterns in a renal tumor, and when it does not fit into a single pure category, the designation of RCC, unclassified is justified. However, we know now that tumors with clear cell cytology and with papillary areas, particularly in younger patients, should raise the possibility of a translocation-associated RCC.
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 Case 3 • Immunohistochemical stain for TFE-3 was strongly positive, confirming it- • “RENAL CELL CARCINOMA ASSOCIATED WITH XP11.2 TRANSLOCATION” • These carcinomas are defined by different translocations involving chromosome Xp11.2, all resulting in gene fusions involving the TFE3 gene. The new WHO classification (2004) has also recognized these tumors as a distinct entity. Among many translocations, t(X;17)(p11.2;q25) and t(X;1)(p11.2;q21) are the commonest, the former resulting in ASPL-TFE3 fusion, and the later in PRCC-TFE3 fusion. The ASPL-TFE3 fusion is similar to that seen in Alveolar Soft Part Sarcoma, with the exception that in the renal tumors the translocation is balanced, whereas it is unbalanced in Alveolar Soft Part Sarcoma. Translocations of TFE3 gene have been shown to result in aberrant overexpression of TFE3 proteins, resulting in strong nuclear immunoreactivity with the antibody against TFE3.
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 TFE-3 IMMUNOSTAIN
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 Case 3 • These carcinomas predominantly affect children and young adults, but cases have been recognized among older patients. • While the most distinctive feature of these tumors is the papillary architecture with clear cell cytology, solid and nested architecture and eosinophilic cytology is not infrequent. • Because of the rarity of recognized cases, their biologic behavior is not clear yet. However, some tumors, particularly those with t(X;17), tend to present with high tumor stage, and occasionally behave in an aggressive manner.
Slide Seminar- IV : Pathology of Renal Tumors - Case 3 End of Case 3
