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Year : 2001  |  Volume : 11  |  Issue : 2  |  Page : 97-99
Castleman disease involving perinephric space in a known case of carcinoma of cervix

MD Oswal Cancer Treatment and Research Foundation, Ludhiana, Punjab, India

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How to cite this article:
Shrimali R, Jain S, Garg L, Setia V, Gupta A, Gupta A. Castleman disease involving perinephric space in a known case of carcinoma of cervix. Indian J Radiol Imaging 2001;11:97-9

How to cite this URL:
Shrimali R, Jain S, Garg L, Setia V, Gupta A, Gupta A. Castleman disease involving perinephric space in a known case of carcinoma of cervix. Indian J Radiol Imaging [serial online] 2001 [cited 2021 Feb 26];11:97-9. Available from:

Castleman disease is a rare lymphoproliferative condition. It was first described in 1956 [1]. It is known as angiofollicular lymph node hyperplasia, giant lymph node hyperplasia and less frequently as lymphoid hamartoma, angiomatous lymphoid hamartoma, benign lymphoma or follicular lymphoreticuloma [2]. Although the mediastinum is considered to be the most common site of involvement, other sites have also been described. We present Castleman disease involving the perinephric space in a case of carcinoma of cervix (Stage I B).

A previously healthy thirty-four-years old multiparous woman presented with chief complaints of irregular bleeding per vaginum. On per vaginal examination a friable ulceroproliferative mass was seen involving the cervix. Biopsy from the lesion revealed a keratinizing squamous cell carcinoma.

Contrast enhanced CT was performed as part of routine metastatic work-up. In addition to the cervical lesion (cervical mass) the CT study revealed a well delineated, rounded, soft tissue attenuation mass with a few small hypodense areas in it, in the right perinephric space. The lesion was abutting the lateral aspect of the inferior pole of the right kidney. A few linear densities consistent with peritumoral vessels were discernible in the adjoining fat; these were easily appreciable in the region inferior to the lesion. The mass was also abutting the ascending colon and inferior portion of the right lobe of the liver. The right adrenal gland was normal and no enlarged retroperitoneal lymph nodes were seen. An umbilical omental hernia was also incidentally noted. A provisional diagnosis of an exophytic renal tumor was made. In view of the provisionally diagnosed carcinoma of cervix a differential diagnosis of perirenal metastatic deposit was also considered.

Wertheim's hysterectomy as well as incision of the perinephric mass were planned. On surgery, a highly vascular mass was found in the right perinephric space. It was adherent to the lower pole of the right kidney. This mass was completely resected. On histopathological examination it was diagnosed as Castleman disease.

Localized Castleman disease is a benign condition. It is most often seen as a solitary mass in the mediastinum (70%). However, the disease may be seen in any other area of the body where lymph nodes are found: lung, neck, axilla retroperitoneum, mesentery and pelvis [3]. The abdominal form is rare, it is seen in 10-17% of cases, the majority being retroperitoneal [1]. An aggressive, widespread form of Castleman disease has also been reported; it often follows a malignant course. Radiologically it mimics lymphoma, with multifocal lymphadenopathy, mass formation, hepatomegaly and splenomegaly [3].

Histopathologically, two forms of the disease are classically recognized. The hyaline vascular type is most frequent (80-90%); it generally affects young people and is usually asymptomatic [3]. It is located in the mediastinum in seventy percent of patients and should be considered in the differential diagnosis of anterior mediastinal masses. [4].

The less common "plasma cell type" is associated in fifty percent of cases with systemic manifestations, such as fever, anemia, elevated ESR and hypergammaglobulinemia. However, both types can coexist and various transitional forms have also been reported [3].

There area various theories concerning the pathogenesis of the disease; a neoplastic anomaly in the cellifferentiation, an immune disorder or a kind of hamartoma. Associations with Hodgkin's disease and other autoimmune conditions have also been described [1].

The radiological features of Castleman disease are non-specific. Early dense contrast enhancement almost equals that of the aorta. On dynamic contrast enhanced CT, presence of calcification is suggestive of the hyaline vascular type. Moderate contrast enhancement is seen in the plasma cell type [5]. Hypertrophied feeding arteries, irregular vascular channels throughout the tumor and a dense homogeneous blush in the capillary phase are angiographic features of the hyaline vascular type [3]. MR findings of Castleman disease include low signal on T1W images and high signal on T2W images. Hypointense linear shadows within the mass on T1W and T2W images may correspond to calcification, fibrous septations or vessels [3].

There have been various case reports on solitary Castleman disease involving the retroperitoneum [2]. On extensive review of literature we could not find any association between carcinoma of cervix and Castleman disease, so we presume that in this case it was an incidental finding.

   References Top

1.Irsutti M, Paul J L, Selves J, Railhae JJ. Castleman disease: CT and MR imaging features of retroperitoneal location in association paraneoplastic pemphigus. Eur. Radiol 1999; 9:1219-1222.  Back to cited text no. 1    
2.Seco Juan L et al. Retroperitoneal Castleman's disease. Surgery. 1992;112: 850-855.  Back to cited text no. 2    
3.Patricio L, et al. Pelvic Castleman disease: CT and MR appearance. J. Comput Assist Tomogr. 1992; 16: 657-659.   Back to cited text no. 3    
4.Ferreiros J, Gomez LN, Mata Isabel MA, Casanova R, Pedrosa CS, Cuevas A. Computed tomography in abdominal Castleman's disease . J. Computer Assist Tomogr.1989; 13: 433-436.  Back to cited text no. 4    
5.Greenstein S, Fishman EK, Kaufman SL, Kadir S, Siegelman SS. Castleman disease of the retroperitoneum: CT demonstration. J. Comput. Assist Tomogr. 1986, 10: 547-548.  Back to cited text no. 5    

Correspondence Address:
R Shrimali
MD Oswal Cancer Treatment and Research Foundation, Ludhiana, Punjab
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Source of Support: None, Conflict of Interest: None

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