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Indian Journal of Cancer, Vol. 46, No. 1, January-March, 2009, pp. 75-76 Letter To Editor Castleman's disease: A rare diagnosis in retroperitoneum Shahi KS, Bhandari G, Hota PK Department of Surgery, U.F.H.T, Medical College, Haldwani, Nainital Code Number: cn09017 Dear Sir, Castleman′s diseases is a rare benign disorder named after the pathologist who first described this clinical entity. We describe here a case of retroperitoneal peri-renal Castleman′s disease. A 22-year-old woman with no significant past medical history presented with complaints of epigastria discomfort and dull aching pain in right lumbar region for six months. Physical examination revealed a mass in right upper quadrant on deep palpation. Routine hematological profile and blood biochemistry were normal. Patient was HIV 1-2 negative. Chest and Abdominal roentgenograms were normal. Abdominal ultrasonography revealed a well-defined solid hypoechoic mass lesion 5 x 8 x 4.3 cm in size just inferior to level of head of pancreas anterior to inferior vena cava causing pressure effect on inferior vena cava.Vascularity was noted in the lesion on color Doppler study. Computed Tomography showed 5.5 x 4.8 x 4.6 cm well defined isodense mass lesion anterior to inferior vena cava in the region of uncinate process of pancreas causing pressure effect on inferior vena cava and left renal vein, uniformly enhancing on contrast study. Although a definitive pre-operative diagnosis could not be made surgical excision was planned. At laparotomy a 8 x 7.5 cm oval mass with thick fibrous capsule was found which was compressing inferior vena cava medially and renal vessels inferiorly. It was also pushing head of pancreas anteriorly, superiorly it was medial to adrenal gland and its capsule was adherent to the liver. Mass was excised en bloc . The cut surface of the mass was grayish and lobular with few grey-brown areas. Pathological diagnosis was Castleman′s disease of hyaline vascular type. Patient had a smooth post operative course and is doing well without any signs of recurrence after eighteen months of surgery. Castleman′s disease is a rare lymphoid disorder. Also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia is a non-clonal disease of lymph nodes characterized by lymphoid and vascular proliferation. It is generally seen in adults usually prior to age of 30. It has also been reported in children and there is no sex predilection for the disease. Castleman′s disease has been classified as unicentric localized form and multicentric generalized form based on clinical and radiological findings. Based on histopathological findings, it is classified into hyaline vascular, plasmacytic and mixed cellularity types. The most common subtype of Castleman′s disease is hyaline vascular type which tends to be localized in one lymph node. It presents as a slow growing mass, generally asymptomatic; symptoms if present are due to a mass effect of bulky lymphadenopathy. Localized form of Castleman′s disease is seen predominantly in the mediastinum, however it has been found in abdomen, neck, axilla, inguinal region and in almost every lymph node basin. [1] One hundred and twenty two cases of localized retroperitoneal Castleman′s disease have been reported in world literature out of which 20% were in peri-renal region as in our case. Nearly all these lesions were derived from lymph node tissues. [2] Clinical presentation of retroperitoneal localized Castleman′s disease can be associated with symptoms related to compression of adjacent organs like, post-prandial discomfort, anorexia, vomiting, weight loss, urinary retention and abdominal or lumbar pain. [3] The differential diagnosis in a reproperitoneal localized Castleman′s disease is mainly malignant neoplasm because of hyperavascularity and the fact that 80% of the reproperitoneal tumors are malignant. [4],[5] The standard treatment of localized Castleman′s disease is surgical excision which is curative when resection is en bloc . The prognosis is excellent with a five-year survival of 100%. Although retroperitoneal Castleman′s disease is uncommon we should consider this tumor in differential diagnosis of retroperitoneal mass. A better understanding of this disease would avoid unnecessary extensive surgery for a benign condition. References
Copyright 2009 - Indian Journal of Cancer |
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