Adult clear cell sarcoma of the kidney: A case report

Kural, Ali Rıza; Önal, Bülent; Özkara, Hamdi; Cakarir, Cansel; Ayan, İnci̇; Ağaoğlu, Fulya Yaman

doi:10.1186/1471-2490-6-11

Cited by 9 publications

(4 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Treatment of CCSK remains controversial, and the optimal treatment is unknown. A combination of chemotherapy regimens (cyclophosphamide, doxorubicin, actinomycin D, and vincristine), and radical nephrectomy with or without radiation is used [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Clear cell sarcoma of the kidney with inferior vena cava thrombus: a case report

et al. 2022

View full text Add to dashboard Cite

Background Clear cell sarcoma of the kidney is an uncommon pediatric renal malignant neoplasm that is typically characterized in 2–3-year-olds by aggressive behavior and late relapses. Our literature review revealed fewer than ten previously reported cases of CCSK with inferior vena cava thrombus, with only five in the pediatric age group. Case presentation We report the case of a 14-year-old Syrian girl who complained of mild pain in the left lumbar region pain with hematuria. On physical examination, a mass was palpated in the left flank. Abdominal ultrasonography revealed a left renal mass (7 × 5 × 2 cm3), associated with dilatation of the left renal vein. Contrast abdominal computed tomography showed a mass measuring 7 × 5 × 3 cm3 with the presence of thrombus extending into the inferior cavity down to the right atrium that was initially diagnosed as Wilms’ tumor. Radical right nephrectomy with excision of the thrombus was undertaken. Histological immunostaining revealed a diagnosis of the tumor as clear cell sarcoma with vascular tumor thrombus extending to the inferior vena cava. Conclusion Clear cell sarcoma and Wilms’ tumor are similar in terms of typical age of appearance, clinical features, and histopathology, but with different methods of treatment and prognosis. The differential diagnosis of such masses is thus very important. We present the case of a patient with clear cell sarcoma with unusual age, with complete removal of the thromboses in the inferior vena cava and the right atrium.

show abstract

Section: Discussionmentioning

confidence: 99%

Clear cell sarcoma of the kidney with inferior vena cava thrombus: a case report

et al. 2022

View full text Add to dashboard Cite

show abstract

“…However, it is classified separately from WT because of several distinctive features. It usually tends to metastasize to bones being different from WT [4].…”

Section: Discussionmentioning

confidence: 99%

Surgical management of clear cell sarcoma of the kidney with tumor thrombus in the inferior vena cava and lung metastasis

Nazlı¹

2014

Diclemedj

View full text Add to dashboard Cite

ÖZETBöbreğin clear hücreli sarkomu (BCHS) çocukluk çağının nadir bir renal tümörüdür. Çocuklarda daha yaygın görülürken, yetişkinlerde oldukça nadir görülür. BCHS'da tümör trombüsü bazen inferior vena kavaya ilerleyebilir. Burada biz inferior vena kavaya yayılan vasküler tumor trombüslü 18 yaşındaki BCHS'lu nadir vakada kardiyopulmoner bypass altında ve kardiyak arrest yapılmaksızın renal kitlenin, vasküler tümör trombüsünün ve pulmoner metastazın başarılı olarak çıkarılmasını tanımladık.Anahtar kelimeler: Böbreğin clear hücreli sarkomu, inferior vena kava, tümör trombüs, kardiopulmoner bypass ABSTRACT Clear cell sarcoma of the kidney (CCSK) is a rare renal neoplasm of childhood. While CCSK is more common in children, it is extremely rare in adults. In CCSK, a tumor thrombus is sometimes formed in the inferior vena cava. Herein, we describe a rare case of an 18-year-old female with CCSK with vascular tumor thrombus extending to the inferior vena cava that was managed successfully by excision of the renal mass and vena caval thrombus, and pulmonary metastasectomy using cardiopulmonary bypass without cardiac arrest.

show abstract

“…Clear cell sarcoma of the kidney (CCSK) is an uncommon malignant tumor of uncertain histogenesis that occurs most commonly in childhood [ 1 ]. Only rare cases have been reported in adults [ 2 ]. It represents 4-5% of primary renal neoplasms and is known for its aggressive behavior, tendency for recurrence, and metastasis to bone [ 3 – 6 ].…”

Section: Introductionmentioning

confidence: 99%

TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature

Naous

2018

Case Reports in Pathology

View full text Add to dashboard Cite

Clear cell sarcoma of the kidney (CCSK) is an uncommon malignant tumor of uncertain histogenesis that occurs most commonly in childhood. Histologically, CCSK can mimic myxoid variant of synovial sarcoma (SS); however, the double positivity for CD99 and TLE1 in SS helps in excluding CCSK. Herein, we report a rare case of CCSK arising in the left kidney of a 3-year-old girl. The mass grossly measured 9.5 cm in maximum dimension and histologically showed the classic arborizing fibrovascular septae and background myxoid matrix that is usually seen in CCSK. The tumor however was double positive for CD99 and TLE1 which made it difficult to discriminate it from the myxoid variant of SS based on histopathological examination and immunophenotype alone, and genetic analysis for SYT gene rearrangement was required to reach a definitive diagnosis. Although there have been previous case reports of CCSK with positive expression of CD99 and negative TLE1, to our knowledge, this is the first case of CCSK expressing both CD99 and TLE1.

show abstract

Adult clear cell sarcoma of the kidney: A case report

Cited by 9 publications

References 5 publications

Clear cell sarcoma of the kidney with inferior vena cava thrombus: a case report

Clear cell sarcoma of the kidney with inferior vena cava thrombus: a case report

Surgical management of clear cell sarcoma of the kidney with tumor thrombus in the inferior vena cava and lung metastasis

TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature

Contact Info

Product

Resources

About