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Year : 2018  |  Volume : 2  |  Issue : 4  |  Page : 96-98

Unicentric hyaline vascular type of castleman disease of the renal hilum with diagnostic dilemma: A case report and review of literature

1 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Urology, Kalinga Hospitals Limited, Bhubaneswar, Odisha, India

Date of Web Publication26-Dec-2018

Correspondence Address:
Dr. Amit Kumar Adhya
Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/oji.oji_36_18

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Castleman disease (CD) represents a morphologically distinct variety of benign proliferation of lymphoid tissue. Renal hilum involvement is rare and may be confused with renal malignancy or granulomatous diseases. Here, we report a rare case of unicentric CD of renal hilum. A 56-year-old male patient presented with flank pain and voiding symptoms. Contrast-enhanced computed tomography scan showed a left renal hilar tumor mimicking a renal malignancy which led to nephrectomy. Our case is the first report of the unicentric hyaline vascular type of CD of the renal hilum reported from India. Awareness of such cases is important for radiologists and surgeons to prevent the misdiagnosis of a renal malignant tumor and to avoid nephrectomy.

Keywords: Castleman disease, hyaline vascular, malignancy, renal hilum, unicentric

How to cite this article:
Adhya AK, Pradhan MR. Unicentric hyaline vascular type of castleman disease of the renal hilum with diagnostic dilemma: A case report and review of literature. Oncol J India 2018;2:96-8

How to cite this URL:
Adhya AK, Pradhan MR. Unicentric hyaline vascular type of castleman disease of the renal hilum with diagnostic dilemma: A case report and review of literature. Oncol J India [serial online] 2018 [cited 2020 Jun 1];2:96-8. Available from: http://www.ojionline.org/text.asp?2018/2/4/96/248532

  Introduction Top

Castleman disease (CD) is an uncommon morphologically distinct type of benign lymphoproliferative disorder characterized by hyperplasia of lymphoid follicles. It can develop in the lymph node at single or multiple sites throughout the body and hence can be called unicentric or multicentric, respectively. The etiology of CD is not clear. The symptoms are usually due to the mass effect of the enlarged lymph node over the adjacent tissue.[1] The multicentric disease may be linked to human herpes virus (HHV)-8 having systemic presentations such as “B” symptoms and have abnormally high levels of interleukin-6 production.[2] Resection of the mass is the curative treatment for the unicentric CD. Whereas, the multicentric disease has no established definitive treatment.[1]

Involvement of the genitourinary tract is rare for the disease and kidney involvement is rarer.[1],[3] Here, we report a case of CD affecting the hilum of the kidney with preoperative diagnostic dilemma.

  Case Report Top

A 56-year-old male presented with voiding lower urinary tract symptoms, nausea, discomfort in the left flank and loss of appetite for 6 weeks. Physical examination was found to be normal. Routine hemogram and the kidney function tests were within normal limits. Both clinical history and laboratory data showed no evidence of any immunodeficiency. Cystoscopy examination revealed grade 2 prostatic enlargement without any bladder pathology. Contrast-enhanced computed tomography (CECT) scan of abdomen and pelvis showed a single well defined mildly enhancing soft-tissue mass of size approximately 6 cm × 4 cm in the hilum of the left kidney [Figure 1]a. Due to almost similar enhancement as that of the renal parenchyma, it was not possible to decide whether the mass was involving the renal parenchyma or confined to the renal sinus only. There was no abdominal lymphadenopathy. The ureter and urinary bladder appeared normal. With the above radiological findings, the possibility of a malignant neoplasm such as transitional cell carcinoma (TCC) or lymphoma was suspected. Further radiological investigations ruled out any presence of distant metastasis. Nephrectomy was done, and the specimen was sent for histopathological examination. On gross examination, the kidney was of normal size with intact renal capsule and was easily stripped off. The renal surface was unremarkable. On slicing, a well-circumscribed fleshy tumor measuring 5 cm × 5 cm was found at the renal hilum. The tumor was well circumscribed and completely within the hilar fat. The renal pelvis was not involved by tumor; although at one place, it appeared adherent to the tumor [Figure 1]b. On microscopic examination, there was an encapsulated tumor within the fat of the renal hilum without attachment to the kidney or renal pelvis. The tumor showed a lymphoid appearance. The lymphoid cells were arranged in concentric manner around central hyalinized blood vessels. Intervening areas show an increase in dendritic cells and plasma cells (PCs) [Figure 1]c and [Figure 1]d. Based on these features, a diagnosis of CD was made. Now, the patient is on close follow-up in urology clinics without any evidence of recurrence of the disease at 24 months of follow-up.
Figure 1: (a) Contrast-enhanced computed tomography scan of abdomen on coronal section showing a well-defined mild enhancing soft-tissue mass of size around 6 cm × 4 cm in the left side renal hilum (b) Gross specimen showing a well circumscribed, fleshy tumor measuring 5 cm × 5 cm at the hilar area of kidney (c and d) Lymphoid cells arranged in concentric manner around central hyalinized blood vessels and intervening areas show increase in the dendritic cells and scattered plasma cells (H and E, ×100)

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  Discussion Top

CD is also known as angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, and angiomatous lymphoid hyperplasia.[2] It was first described by Benjamin Castleman in 1956 as localized mediastinal lymph node hyperplasia.[4] Thorax followed by neck are the common sites of occurrence for CD consisting of 67%–70% and 14%–40%, respectively.[5] Histologically, CD has been subclassified as hyaline vascular (HV) type, PC type, mixed type, and plasmablastic type. HV is the most common type consisting of around 90% of CD and mostly presents as a single unicentric mass in young people harboring a favorable prognosis, whereas the PC type is less common, mostly multicentric and associated with worse prognosis.[2],[6]

CD with renal primary is an extremely rare entity with only few cases were reported in the literature and most of the cases were of PC type with HV type being rarer.[1],[5] The tumors were incidentally detected in many patients who underwent radiological evaluation of the abdomen for symptoms unrelated to renal disease.[2],[3],[7] However, our patient was HV type of CD involving renal hilum and had urinary symptoms and flank pain due to the large tumor size.

Radiological findings of CD confined to renal sinus have been rarely described. Nishie et al. reported three cases of CD involving renal sinus having five masses (one case: unilateral and two cases: bilateral masses), and found mixed or PC type of histology with the range of diameter for masses of 3.0–4.5 cm. They found all the masses were homogeneous appearance with attenuation slightly higher on unenhanced CT and slightly lower on the early- and delayed-phase of CECT than renal parenchyma. Mild hydronephrosis was found in all the kidneys with mass as blunting of the calices with no filling defect in the collecting system. Lack of filling a defect in the collecting system may suggest a noninvasive tumor. Whereas, our case was unicentric HV type of CD but with the mild enhancing appearance and the tumor was around 6 cm × 4 cm of size on CECT scan but without hydronephrosis. Preoperative magnetic resonance imaging (MRI) and angiography are the part of the investigations for the renal hilum masses which was not done in our case. PC type of CD is less vascular and shows mild enhancement with hypointensity on T2-weighted MRI, whereas marked enhancement and hyperintensity has been detected in HV type which is highly vascular. All the cases of CD involving renal sinus reported by Nishei et al. and Kim et al. showed homogeneous and hypointensity appearance on T2weighted MRI.[5],[8] With these findings, Kim et al. state that mild enhancement and hypointensity on T2-weighted MRI may be helpful for the diagnosis of PC type of CD.

TCC, lymphoma, and IgG4-related disease are the possible differential diagnoses for renal hilar masses. In addition granulomatous lesions such as tuberculosis and sarcoidosis, sarcomas, and metastasis may also be considered.[5],[8] Radiologically, TCC can be a sessile or flat solid mass in the renal calyx and/or pelvis, focal renal pelvic wall thickening, or an infiltrating mass. However, TCC had more obstruction of the collecting system in comparison to CD.[8] Lymphoma may involve renal parenchyma and sinus and has relatively iso-or hypointense in T2-weighted sequences. Due to the pliable nature of lymphoma, mild hydronephrosis may result. However, the presence of retroperitoneal adenopathy in lymphoma may help to distinguish it from Castleman's disease.[8],[9] Renal involvement of IgG4-related disease also shows hypointense on T2-weighted images. However, the synchronous or metachronous involvement of other organs such as pancreas and high serum IgG4 concentrations with favorable response to steroid therapy helps to distinguish it from CD.[10] Most of the renal hilum CD reported were PC type. Whereas, HV type of CD involving renal hilum is extremely rare and radiological investigations for such variant was not clearly described in the literature.

In almost all the cases, radiological features were mistaken for a primary renal tumor and patient was subjected to nephrectomy. The diagnosis of CD was made postoperatively on pathology evaluation of the tumor as in our case.[8] Hence, a systematic review of all the cases of CD described till date is necessary to avoid unnecessary nephrectomy. Follow-up of the reported cases did not show any recurrence of the tumor, and hence, surgical excision is adequate treatment for such cases. Radical nephrectomy is not recommended, and the tumor may be managed by transperitoneal laparoscopic removal if the tumor is in the hilum of the kidney and not involving the renal parenchyma.[1]

In summary CD of renal hilum is extremely rare. It may be confused with a primary renal tumor for which radical nephrectomy is commonly performed. Complete clinical and radiological correlation along with the suspicion of the disease are the key factors for its diagnosis and avoidance of unnecessary extensive surgery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Radfar MH, Pakmanesh H, Torbati P. Castleman disease presenting as renal hilar mass. J Endourol Case Rep 2015;1:54-5.  Back to cited text no. 1
Mah NA, Peretsman SJ, Teigland CM, Banks PM. Castleman disease of the hyaline-vascular type confined to the kidney. Am J Clin Pathol 2007;127:465-8.  Back to cited text no. 2
Koh Y, Kinjo T, Nonomura D, Yamamoto Y, Yoneda S, Nomura H, et al. Castleman's disease of the kidney: A case report. Hinyokika Kiyo 2014;60:129-32.  Back to cited text no. 3
Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer 1956;9:822-30.  Back to cited text no. 4
Nishie A, Yoshimitsu K, Irie H, Aibe H, Tajima T, Shinozaki K, et al. Radiologic features of Castleman's disease occupying the renal sinus. AJR Am J Roentgenol 2003;181:1037-40.  Back to cited text no. 5
Kwon JH, Min SK, Shin MK, Lee YS, Lee YG, Ko YH. Hyaline vascular Castleman disease involving renal parenchyma and a lymph node: A case report. Korean J Pathol 2012;46:79-82.  Back to cited text no. 6
Ryu JH, Oh JW, Kim KH, Choi JI, Ryu KH, Kim YJ, et al. Castleman disease misdiagnosed as a neoplasm of the kidney. Korean J Urol 2009;50:413-6.  Back to cited text no. 7
Kim TU, Kim S, Lee JW, Lee NK, Jeon UB, Ha HG, et al. Plasma cell type of Castleman's disease involving renal parenchyma and sinus with cardiac tamponade: Case report and literature review. Korean J Radiol 2012;13:658-63.  Back to cited text no. 8
Sheth S, Ali S, Fishman E. Imaging of renal lymphoma: Patterns of disease with pathologic correlation. Radiographics 2006;26:1151-68.  Back to cited text no. 9
Takahashi N, Kawashima A, Fletcher JG, Chari ST. Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 2007;242:791-801.  Back to cited text no. 10


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