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2022, Volume 38, Number 2, Page(s) 148-152
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DOI: 10.5146/tjpath.2021.01541 |
Atrophic Kidney-Like Lesion – Case Report of A Provisional Entity with Brief Review of Literature |
Balamurugan THIRUNAVUKKARASU1, Saket SINGH2, Aditya Prakash SHARMA2, Amanjit BAL1 |
1Departments of Histopathology, Post Graduate Institute of Medical Education & Research (PGIMER), CHANDIGARH , INDIA
2Departments of Urology, Post Graduate Institute of Medical Education & Research (PGIMER), CHANDIGARH , INDIA |
Keywords: Atrophic kidney-like lesion, Thyroid-like follicular carcinoma, Kidney |
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Atrophic kidney-like lesion is a recently recognized entity, post 2016 World Health Organization Classification of tumors of the urinary
system. The behavior of this tumor is not fully known as only a handful of cases with limited follow-up are available. This entity closely mimics
thyroid-like follicular carcinoma of the kidney, which has different prognosis.
We report a case of incidentally detected atrophic kidney-like lesion in an elderly gentleman who had urothelial carcinoma of the urinary bladder
with a brief review of literature.
Atrophic kidney-like lesion and urothelial carcinoma of the urinary bladder association has not been reported in the literature. |
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Atrophic kidney-like lesion (AKLL) is a recent entity
described post 2016 World Health Organization (WHO)
classification of tumors of the urinary system. With the
available limited follow-up data, this is considered as a
benign renal neoplasm with indolent behavior. Owing
to its follicular architecture, it closely resembles thyroidlike
follicular carcinoma of the kidney (TFRCC) 1. The
distinction between the two is essential as the latter has
chromosomal alterations in the form of gains and losses
and an aggressive behavior with metastatic potential 2,3. |
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Abstract
Introduction
Case Presentation
Disscussion
References
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A 71-year-old gentleman was a known case of recurrent
low-grade urothelial carcinoma for 9 months for which
he had undergone transurethral bladder tumor resection
thrice. On routine surveillance, contrast enhanced
computed tomography (CECT) abdomen showed a wellcircumscribed
tumour measuring 2.6x2.4cm in the midthird
region of the right kidney with focal extension into
the upper pole. The kidney measured 5.5cm in length with
indistinct corticomedullary junction (Figure 1A,B). The
patient underwent simple nephrectomy for the lesion. On
gross examination, the renal capsule was intact, and the
cut surface showed a well-demarcated tan brown lesion
measuring 3x2.8x2.5cm in the mid-third region. (Figure
1C). The renal sinus and ureter were unremarkable. On
microscopy, the lesion was well demarcated from the renal parenchyma by a thin fibrous capsule (Figure 2A).
The lesion was composed of compact tubules lined by
flattened and atrophic epithelium interspersed by cyst-like
follicles. Many of the follicles were filled with pale to dense
eosinophilic material detached from the epithelium (Figure
2B,C). The stroma between the follicles showed collagen
deposition, few atrophic tubules, and capillaries. Focal
amorphous calcific areas were also noted (Figure 2D). No
atypical features like mitosis, necrosis or high nuclear grade
areas were noted.
The differential diagnoses considered were atrophic
kidney-like lesion, thyroid follicle-like renal cell carcinoma,
metastatic follicular carcinoma of the thyroid, and welldifferentiated
neuroendocrine tumour (carcinoid). A panel
of immunohistochemistry was performed that included
PAX8 (Cell Marque, RTU, clone MRQ-50), CK7 (Cell
Marque, 1:300, clone OV-TL12/30), WT-1 (Dako, 1:50,
clone 6F-H2), Synaptophysin (Cell Marque, 1: 200, Clone
MRQ-40), CD117 (Dako, 1:500, clone A4502), and CD10
(Cell Marque, 1:30, clone 56C6). The cells were diffusely
positive for PAX-8 (Figure 3A), and CK7 (Figure 3B),
while negative for CD10 (Figure 3C), TTF-1 (Figure 3D),
Synaptophysin, WT-1 and CD117. The renal sinus, pelvis
and ureter were free. No extracapsular invasion was noted.
Adjacent renal parenchyma showed preserved glomeruli
and non-atrophic tubules. Based on morphology and
immunophenotyping, an ‘Atrophic kidney-like lesion’ was
diagnosed; Tumor stage – T1a; World Health Organization/International Society of Urological Pathology (WHO/
ISUP) Nuclear grade 1. There were no post-surgical
complications. The patient has been on regular follow-up
till date without any recurrence or distant metastasis.
Click Here to Zoom |
Figure 1: A, B) Contrast-enhanced CT
abdomen showed a well-circumscribed
tumour nodule in the upper pole in the
background of a small kidney
C) Cut surface shows a well-demarcated
tan brown colored tumour measuring
3x2.8x2.5cm in the mid-third region. |
Click Here to Zoom |
Figure 2: A) Tumour composed
of multiple tightly packed varying
sized follicles separated from
native kidney by a thin capsule
(arrow pointed) (H&E, 40x)
B) The follicles are filled with
dense eosinophilic colloid-like
secretions (H&E, 100x)
C) The lumen is filled with pink
eosinophilic material and focal
macrocystic degeneration (H&E,
40x) D) The follicles are lined by
flattened to cuboidal epithelium
with occasional amphophilic
calcific deposits (H&E, 200x). |
Click Here to Zoom |
Figure 3: A) The tumour (follicular) cells are positive for PAX-8; B) CK7 Positive; C) CD10 Negative D) TTF-1 Negative (Immunoperoxidase,
200x) |
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Top
Abstract
Introduction
Case Presentation
Disscussion
References
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The nomenclature “Atrophic kidney-like lesion (AKLL)”
has been used interchangeably in the literature with
“atrophic kidney-like tumour” and “atrophic kidney-like
renal cell carcinoma” 4,5. This was originally described
by Hes et al in 2014 as a separate entity and its clonal nature
was proven in a series of 3 cases 1. Atrophic kidneylike
lesion was initially considered under Thyroid-like
follicular carcinoma of the kidney (TFRCC) 5. Following
the original description, many cases were reviewed and
described as reports and case series, of which few were
initially diagnosed as TFRCC 6,7. The pathogenesis of
AKLL is compared with that of glomerulocystic disease
as the cells lining the cysts expressed WT-1, which is a
marker of podocyte differentiation 8. At low power, both the tumors impart a follicular architecture. However, the
distinguishing feature between AKLL and TFRCC can be
appreciated on higher magnification. In TFRCC, the cells
lining the follicles are cuboidal with abundant eosinophilic
cytoplasm, high nuclear grade, and prominent nucleoli,
resembling a proper follicular neoplasm of the thyroid
whereas in AKLL the cells are flat/atrophic to low cuboidal
9,10.
Distinction of atrophic kidney-like lesion from the end
stage renal disease induced by chronic pyelonephritis is
important. Chronic pyelonephritis with thyroidization
also shows atrophic tubules with hyaline casts mimicking
the neoplasm. The important distinguishing point is the
presence of a well-defined capsule, lack of inflammation,
and absence of glomeruli in-between the lesion. In the
present case, the background renal parenchyma did not
show features of chronic pyelonephritis. The differential
diagnosis of AKLL includes metastatic follicular carcinoma
of the thyroid, metanephric adenoma, multilocular
cystic renal neoplasm of low malignant potential, and tubulocystic renal cell carcinoma 2,11. Metastatic
follicular carcinoma of the thyroid shows TTF-1 expression,
whereas metanephric adenoma shows tightly packed
tubules lined by uniform cuboidal cells with occasional
presence of papillary structures and diffuse positivity for
WT1. Multilocular cystic renal neoplasm of low malignant
potential shows cystic areas lined by clear cells positive for
CD10. Tubulocystic renal cell carcinoma shows uniformly
dilated cystic spaces intervened by fibrotic to hyalinized
stroma. The cystic spaces are lined by cells with significant
nuclear abnormalities and variable hobnailing.
To the best of our knowledge, 13 cases of AKLL have been
reported in the English literature (Table I). Many of the
cases were reviewed in the previously published cases of
TFRCC. This lesion is usually reported in young patients
with no major gender predilection. Age distribution of
AKLL is from 9 to 58 years with a median age of 30.7 years.
No specific genetic alterations have been reported yet 8.
Classification of renal tumors is constantly evolving, and
many new entities are being added and existing entities
are being reclassified. Atrophic kidney-like lesion is a new
entity with indolent behavior and described using different
names. The Genitourinary Pathology Society (GUPS) has
placed this lesion under “provisional entity” requiring
more data for validation 4. The present case had thin
well-defined capsule, occasional amorphous calcification
with PAX8 and CK7 positivity. Features like high-grade
nuclei, mitosis or necrosis were not seen. This index patient
had completed treatment for recurrent low-grade papillary
urothelial carcinoma of the urinary bladder and the renal
lesion was incidentally detected on routine radiological
surveillance. This association of AKLL with urothelial
carcinoma has not been reported before.
Click Here to Zoom |
Table I: Published cases of atrophic kidney-like lesion in the literature |
CONFLICT of INTEREST
The authors declare no conflict of interest.
AUTHORSHIP CONTRIBUTIONS
All the authors were involved in conception and design of
the work. BM and SS were involved in data collection and
writing. AB and APS were involved in analysis of data and
approval. |
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Abstract
Introduction
Case Presentation
Discussion
References
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1) Hes O, de Souza TG, Pivovarcikova K, Grossmann P, Martinek P,
Kuroda N, Kacerovska D, Svajdler M, Straka L, Petersson F, Hora
M, Michal M. Distinctive renal cell tumor simulating atrophic
kidney with 2 types of microcalcifications. Report of 3 cases. Ann
Diagn Pathol. 2014;18:82-8.
2) Jung SJ, Chung JI, Park SH, Ayala AG, Ro JY. Thyroid follicular
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2006;30:411-5.
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Y. Thyroid-like follicular carcinoma of the kidney in a patient
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7) Berens S, Vogt P, Alkadhi H, Berger N, Moch H. Thyroid-like
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M, Nguyen JK, Troxell ML, Przybycin CG, Magi-Galluzzi C,
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9) Amin MB, Gupta R, Ondrej H, McKenney JK, Michal M,
Young AN, Paner GP, Junker K, Epstein JI. Primary thyroidlike
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histologically distinctive adult renal epithelial neoplasm. Am J
Surg Pathol. 2009;33:393-400.
10) Muscara MJ, Simper NB, Gandia E. Thyroid-like follicular
carcinoma of the kidney. Int J Surg Pathol. 2017;25:73-7.
11) Amin MB, MacLennan GT, Gupta R, Grignon D, Paraf F,
Vieillefond A, Paner GP, Stovsky M, Young AN, Srigley
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of renal cell carcinoma. Am J Surg Pathol. 2009;33:384-92. |
Top
Abstract
Introduction
Case Presentation
Discussion
References
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Copyright © 2022 The Author(s). This is an open-access article published by the Federation of Turkish Pathology Societies under the terms of the Creative Commons Attribution License that permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited. No use, distribution, or reproduction is permitted that does not comply with these terms. |
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