Localised renal Langerhans cell histiocytosis coexisting with unilateral renal clear cell carcinoma

Abstract

Langerhans cell histiocytosis (LCH) is an uncommon group of disorders, which can be either localised or systemic, characterised by abnormal proliferation of monocytes, macrophages and dendritic cells. These disorders represent an aberrant response of myeloid progenitor cells. Bones are the most commonly affected organ but there can be involvement of the skin, lungs, liver and spleen. Renal involvement, however, is rare. LCH is the most commonly seen in children but certain rare forms such as Erdheim-Chester disease can be seen in adults. In this report, we present a case of clear cell renal adenocarcinoma (CCRC) admixed with LCH in a patient with history of smoking and presenting with abdominal pain and heamaturia. Imaging revealed left renal lesion and subsequently left renal nephrectomy was performed with tissue biopsy showing grade 3 clear cell renal cell carcinoma admixed with neoplastic LCH.

Response to: Langerhans Cell Histiocytosis Associated With Renal Cell Carcinoma

“…I read with great interest the Letters to the Editor published recently by Tran and Mekhail1 in the American Journal of Surgical Pathology , in which they reported a new case of Langerhans cell histiocytosis (LCH) associated with clear cell renal cell carcinoma (ccRCC) in response to our recently published series on 7 patients 2. The author’s case together with another case published after our study bring the total number of reported cases to 12 1–3. Moreover, Tran and Mekhail1 reported 2 special features in their case: (1) the younger age of the patient (27 compared with an age range of 39 to 73 y for the remainder), and (2) the concurrent extrarenal manifestation of the LCH involving the vertebral body T9 verified histologically.…”

Langerhans Cell Histiocytosis Associated With Renal Cell Carcinoma

“…Most patients appeared to follow an indolent clinical course as 8 patients merely presented with RCC with intratumoral LCH and no signs of systemic LCH or recurrent RCC at the time of presentation or subsequent followup. 1,[3][4][5] However, clinical follow-up information was available only in 50% of the cases (4/8 cases), and the follow-up time was relatively short with a mean of 29 months (range: 6 to 60 mo). In contrast, approximately one third of the cases displayed a more ominous clinical presentation with clinical signs of systemic LCH preceding, at the time, or after the detection of RCC.…”

“…Although the largest study by Agaimy and colleagues concluded that all RCC involved by LCH are low grade and limited to the renal parenchyma, the RCC in the most recent case report displayed high ISUP/WHO nuclear grade 3 and higher stage with invasion into the Gerota's fascia. 5 The clinical course of patients with LCH associated with RCC is quite unpredictable. Most patients appeared to follow an indolent clinical course as 8 patients merely presented with RCC with intratumoral LCH and no signs of systemic LCH or recurrent RCC at the time of presentation or subsequent followup.…”

“…Interestingly, the left kidney is 3 times more often than the right to be involved by this peculiar phenomenon. Although the largest study by Agaimy and colleagues concluded that all RCC involved by LCH are low grade and limited to the renal parenchyma, the RCC in the most recent case report displayed high ISUP/WHO nuclear grade 3 and higher stage with invasion into the Gerota’s fascia 5…”

“…The clinical course of patients with LCH associated with RCC is quite unpredictable. Most patients appeared to follow an indolent clinical course as 8 patients merely presented with RCC with intratumoral LCH and no signs of systemic LCH or recurrent RCC at the time of presentation or subsequent follow-up 1,3–5. However, clinical follow-up information was available only in 50% of the cases (4/8 cases), and the follow-up time was relatively short with a mean of 29 months (range: 6 to 60 mo).…”