Fat-poor renal angiomyolipoma with prominent cystic degeneration: A case report and review of the literature

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

BACKGROUND

Angiomyolipoma (AML), the most common benign tumor of the kidney, is usually composed of dysmorphic blood vessels, smooth muscle, and mature adipose tissue. To our knowledge, AML with cystic degeneration has rarely been documented. Cystic degeneration, hemorrhage, and a lack of fat bring great challenges to the diagnosis.

CASE SUMMARY

A 60-year-old man with hypertension presented with a 5-year history of cystic mass in his left kidney. He fell 2 mo ago. A preoperative computed tomography (CT) scan showed a mixed-density cystic lesion without macroscopic fat density, the size of which had increased compared with before, probably due to hemorrhage caused by a trauma. Radical nephrectomy was performed. Histopathological studies revealed that the lesion mainly consisted of tortuous, ectatic, and thick-walled blood vessels, mature adipose tissue, and smooth muscle-like spindle cells arranged around the abnormal blood vessels. The tumor cells exhibited positivity for human melanoma black-45, Melan-A, smooth muscle actin, calponin, S-100, and neuron-specific enolase, rather than estrogen receptor, progesterone receptor, CD68, and cytokeratin. The Ki-67 labeling index was less than 5%. The final diagnosis was a fat-poor renal AML (RAML) with prominent cystic degeneration.

CONCLUSION

When confronting a large renal cystic mass, RAML should be included in the differential diagnosis.

Related collections

Most cited references 46

Record: found
Abstract: found
Article: not found

2004 WHO classification of the renal tumors of the adults.

Antonio Lopez-Beltran, Marina Scarpelli, Rodolfo Montironi … (2006)

The recently introduced 2004 World Health Organisation (WHO) classification of the adult renal epithelial neoplasms is meant to replace the previous 1998 WHO classification. The 2004 WHO classification is based on pathology and genetic abnormalities. The description of categories has been expanded to improve their recognition and new diagnostic categories are included. Emphasis has been placed on defining familial renal cancer, carcinoma associated with Xp11 translocations, carcinoma associated with neuroblastoma, multilocular cystic renal cell carcinoma, tubular, mucinous and spindle cells carcinoma; and mixed epithelial and stromal tumour. The potentially aggressive epithelioid angiomyolipoma is recognised. Recognising these categories may have important implications in patients' clinical management.

0 comments Cited 230 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: found

Is Open Access

Tuberous Sclerosis Complex Surveillance and Management: Recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference

Darcy Krueger, Hope Northrup (2013)

BACKGROUND Tuberous sclerosis complex is a genetic disorder affecting every organ system, but disease manifestations vary significantly among affected individuals. The diverse and varied presentations and progression can be life-threatening with significant impact on cost and quality of life. Current surveillance and management practices are highly variable among region and country, reflective of the fact that last consensus recommendations occurred in 1998 and an updated, comprehensive standard is lacking that incorporates the latest scientific evidence and current best clinical practices. METHODS The 2012 International Tuberous Sclerosis Complex Consensus Group, comprising 79 specialists from 14 countries, was organized into 12 separate subcommittees, each led by a clinician with advanced expertise in tuberous sclerosis complex and the relevant medical subspecialty. Each subcommittee focused on a specific disease area with important clinical management implications and was charged with formulating key clinical questions to address within its focus area, reviewing relevant literature, evaluating the strength of data, and providing a recommendation accordingly. RESULTS The updated consensus recommendations for clinical surveillance and management in tuberous sclerosis complex are summarized here. The recommendations are relevant to the entire lifespan of the patient, from infancy to adulthood, including both individuals where the diagnosis is newly made as well as individuals where the diagnosis already is established. CONCLUSIONS The 2012 International Tuberous Sclerosis Complex Consensus Recommendations provide an evidence-based, standardized approach for optimal clinical care provided for individuals with tuberous sclerosis complex.

0 comments Cited 116 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Radical nephrectomy for pT1a renal masses may be associated with decreased overall survival compared with partial nephrectomy.

R Houston Thompson, Stephen A Boorjian, Christine M Lohse … (2008)

We reviewed our surgical experience with small renal tumors, comparing overall survival in patients treated with radical and partial nephrectomy. Using our nephrectomy registry we identified patients with sporadic, unilateral, solitary and localized renal masses 4 cm or less who underwent radical or partial nephrectomy between 1989 and 2003. Patients with a solitary kidney or impaired renal function at presentation were excluded, leaving 648 available for analysis. Overall survival was estimated using the Kaplan-Meier method and associations with death were evaluated using Cox proportional hazards regression. At last followup 146 patients had died of any cause and 502 were alive at a median of 7.1 years. Radical and partial nephrectomy was performed in 290 and 358 patients, respectively. In all patients radical nephrectomy was not significantly associated with death from any cause compared with partial nephrectomy (RR 1.12, p = 0.52). However, there was a significant interaction with age, leading us to stratify our analysis at the median age of 65 years. In 327 patients younger than 65 years radical nephrectomy was significantly associated with death from any cause compared with partial nephrectomy (RR 2.16, p = 0.02). The increased risk of death persisted after adjusting for year of surgery (p = 0.02), preoperative creatinine (p = 0.03), Charlson-Romano index (p = 0.04), symptoms at presentation (p = 0.02), diabetes at presentation (p = 0.03) and histology (p = 0.02). Our results suggest that, compared with partial nephrectomy, radical nephrectomy is associated with decreased overall survival in younger patients with small renal masses.

0 comments Cited 100 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Contributors

Shi-Qi Lu

Wei Lv

You-Jun Liu

Huan Deng

Journal

Journal ID (nlm-ta): World J Clin Cases

Journal ID (publisher-id): WJCC

Title: World Journal of Clinical Cases

Publisher: Baishideng Publishing Group Inc

ISSN (Electronic): 2307-8960

Publication date (Print): 16 January 2023

Publication date (Electronic): 16 January 2023

Volume: 11

Issue: 2

Pages: 417-425

Affiliations

Medical College, Nanchang University, Nanchang 330006, Jiangxi Province, China

Department of Gastroenterology, West China Hospital, Sichuan University, Chengdu 610044, Sichuan Province, China

Department of Radiology, The Fourth Affiliated Hospital of Nanchang University, Nanchang 330003, Jiangxi Province, China

Department of Pathology, The Fourth Affiliated Hospital of Nanchang University, Nanchang 330003, Jiangxi Province, China. beandeng@ 123456ncu.edu.cn

Author notes

Author contributions: Lu SQ and Lv W carried pathological analyses and drafted the manuscript; Liu YJ analyzed the images; Deng H conceived of this study and drafted the manuscript; and all authors issued final approval for the version to be submitted.

Supported by the National Science Foundation of China, No. 81860490 and 82160546; and the Science Foundation of Jiangxi Province, No. 20202BBG73027.

Corresponding author: Huan Deng, MD, PhD, Professor, Department of Pathology, The Fourth Affiliated Hospital of Nanchang University, No. 133 South Guangchang Road, Nanchang 330003, Jiangxi Province, China. beandeng@ 123456ncu.edu.cn

Article

Other ID: jWJCC.v11.i2.pg417

DOI: 10.12998/wjcc.v11.i2.417

PMC ID: 9850960

PubMed ID: 36686346

SO-VID: 360dbd36-1702-4401-bc97-331d13b60d9d

License:

This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/

History

Date received : 7 October 2022

Date revision received : 16 November 2022

Date accepted : 21 December 2022

Comments

Comment on this article

scite_

Smart Citations

Citing PublicationsSupportingMentioningContrasting

View Citations

See how this article has been cited at scite.ai

scite shows how a scientific paper has been cited by providing the context of the citation, a classification describing whether it supports, mentions, or contrasts the cited claim, and a label indicating in which section the citation was made.