Unicentric Castleman’s Disease: Laparoscopic Approach of a Para-Duodenal Retroperitoneal Mass

Abstract

Patient: Female, 34-year-old Final Diagnosis: Unicentric Castleman’s disease Symptoms: Hematuria Medication:— Clinical Procedure: Laparoscopic approach of a para-duodenal retroperitoneal mass Specialty: Surgery Objective: Rare disease Background: Castleman’s disease is a benign, lymphoproliferative disorder that is extremely uncommon. Multiple classifications have been described; however, the exact etiology remains unknown. Preoperative diagnosis is not common, as imaging cannot distinguish the disease from other processes, and biopsy is insufficient to provide the architecture of the mass, which is necessary for diagnosis. Unicentric retroperitoneal disease has been described, and management includes complete resection of the mass, which is usually curative. Case Report: A 34-year-old previously healthy woman presented with hematuria. Evaluation revelated a retroperitoneal mass that was abutting the duodenum and head of the pancreas. Biopsy failed to provide a diagnosis, so laparoscopic resection was performed. Postoperative diagnosis was consistent with unicentric Castleman’s disease. Conclusions: Castleman’s disease is an uncommon process, and one that is difficult to diagnose. Unicentric Castleman’s disease should always be a differential diagnosis of solitary retroperitoneal masses that are well-demarcated, as treatment can be curative with surgical resection.

Castleman's disease: Laparoscopic resection of unicentric disease in the hepatic hilum: A case report

“…Histopathologically, there are three primary subtypes; hyaline-vascular (70–80%), plasma cell, and mixed [ 4 ]. The hyaline vascular type is the most common and is characterized by follicular hyperplasia and its germinal centers exhibiting capillaries with hyaline matter [ 3 , 5 ]. All three variants present can present clinically with lymphadenopathy or systemic symptoms [ 3 , 6 ].…”

“…UCD is a rare disease with 21 cases per million; it seems to have a slight predisposition with the female population between the third or fourth decades of life [ 2 , 3 , 7 ]. It usually affects the lymphoid tissues of the thorax (70%), neck (15%), abdomen-pelvis (12%), and axilla (3%) [ 5 ]. Mesenteric UCD is rare, with less than 100 cases reported worldwide [ 2 , 3 ].…”

“…As symptoms are vague and non-specific, preoperative diagnosis without imaging studies is extremely difficult; therefore, high clinical awareness is critical [ 3 , 4 , 9 ]. Imaging studies such as CT, positron emission tomography, magnetic resonance imaging, and echography can reveal the location and characteristics of the lymph mass, yet, many tumors including GIST, neurogenic tumors, or ectopic pheochromocytoma, can show similar radiological features making the differential even more challenging [ 4 , 5 , 10 ].…”

“…Final diagnosis is made by pathology; a biopsy by fine-needle aspiration can be helpful; nonetheless, achieving an adequate amount of tissue, the possibility of spreading tumor cells, and the risk of severe bleeding in hypervascular mass should always be considered [ 4 , 13 ]. Even though UCD is not a malignant condition but many dangerous tumors, including Non-Hodgkin lymphoma, paraganglioma, leiomyoma, leiomyosarcoma, fibrosarcoma, and amyloidosis, have been associated with it [ 3 , 5 ]. The differential must also include diseases with lymphadenopathy, including tuberculosis, lymphadenitis, abscess, sarcoidosis, HIV, toxoplasmosis, among others [ 1 , 3 , 6 ].…”

Unicentric form of Castleman´s disease, pitfalls of diagnosis and surgical treatment

“…Nevertheless, further screening is recommended especially at specialized hematologic centers. In contrast, MCD affects multiple lymph nodes all over the body and these patients usually have severe symptoms and are treated by a hematooncologist usually after lymph node harvesting ( 2 , 3 ). One of the key issues in UCD is precise preoperative diagnostics which is indispensable for choosing the right surgical treatment strategy.…”