Castleman Disease (CD) is a rare non malignant lymphoproliferative disorder of uncertain etiology, first reported by Benjamin Franklin in 1954.1 It is also known as giant lymph node (LN) hyperplasia or angiofollicular hyperplasia. Although it can present at any age, its peak incidence is in 3rd and 4th decade.4
It can be classified clinically (on the basis of location) into two types: Unicentric CD (localised disease) and multricentric CD (diffuse or systemic disease). Histopathologically, CD is of two types: hyaline vascular variant (HVV) and plasma cell variant (PCV). UCD is more common (68-98%) than MCD. Further, majority of UCD are HVV, whereas majority of MCD are PCV.4,5
UCD is usually an asymptomatic disease detected incidentally whereas MCD often presents with constitutional symptoms (fever, night sweats, weight loss etc). However, occasionally UCD may present with features of mass effect on adjacent on adjacent organs such as cough, dysphagia, dyspnoea or pain.6
Although CD can arise at any site where lymph node tissue is present, but its most common site is in the thorax (70%). Other sites are abdomen and pelvis (10-15%), neck (10-15%). In the thorax, most common site of CD is the mediastinum. Other intrathoracic sites of UCD are the hilum of lung, pleura, and chest wall.7
Identifying Castleman disease preoperatively is difficult. In the present case, a well-defined posterior mediastinal mass was incidentally detected on CECT chest in an asymptomatic 53-year old female which on preoperative biopsy on histopathology was considered as an inflammatory myofibroblastic tumour. Intra-operatively, the appearance was like that of a malignant tumour. Preoperative contrast imaging (CT/ MRI) may help to differentiate CD from other more common posterior mediastinal masses (neurogenic tumours e.g. schwannoma, neurofibroma, ganglioneuroma). On imaging, CD presents as a well-defined, homogeneous mass; however, the characteristic feature of UCD on CECT is the intense enhancement during arterial phase which decreases during portal venous phase. This intense enhancement is due to hypervascular nature of localised CD (UCD). Hence, multiphase CECT may be performed as the investigation of choice if UCD is suspected.8 Further CT may reveal relation of mass to adjacent organs and major vessels of thorax, helping in surgical planning.
Identifying or suspecting UCD preoperatively may be useful for surgical planning. Localised/Unicentric CD (UCD) is amenable to complete surgical resection which is curative. However, UCD is known to be hypervascular and have dense adhesions with adjacent organs or major vessels2 which may pose a surgical challenge. In such cases, either preoperative embolisation may be done (to reduce vascularity) 9 or mass may be excised on cardiopulmonary bypass (CPB) if dense adhesions with major vessels is present.10 Video-assisted thoracoscopy (VATS) may be associated with less postoperative pain and less hospital stay; however, due to dense adhesions and vascular nature of CD, VATS does not appear to be a lucrative option in CD.10
Prognosis of UCD is good as it behaves as a benign disease and recurrence is rare; hence complete surgical resection is curative. On the other hand, MCD can present as an aggressive lymphoproliferative disease and debulking surgery and/or immunochemotherapy are treatment options in such cases.7