Image of the Month—Diagnosis FREE

Macroscopically, the specimen showed a plaque of 4.5 cm next to the gallbladder neck. Histologically, Richter syndrome with gallbladder localization was observed. Two of 3 lymph nodes close the specimen showed the same histological aspects. Immunophenotypically, the neoplastic cells were intensely positive for Pan-B (MB2) and negative for CD20 and CD30. A considerable amount of T-cell lymphocytes were observed (70% CD3⁺and CD8⁺; 30% CD5⁺) (Figure 2).

Richter syndrome has been defined as the transformation in a high-grade non-Hodgkin lymphoma, prolymphocytic leukemia, Hodgkin disease, or acute leukemia supervening in the course of B-CLL or small lymphocytic lymphoma.³This secondary high-grade lymphoma, usually with diffuse large B-cells, develops in approximately 2.2% to 8% of patients.³It occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same lymphocytic clone population in the course of B-CLL. Clinically, it is often characterized by sudden deterioration of general conditions and development of systemic symptoms such as fever, weight loss, night sweating, and other rare symptoms related to localization of disease. Therapy so far proved unsuccessful, and the overall duration of survival for patients is approximately 5 to 8 months after Richter transformation.³Extranodal localizations are uncommon; the more frequent cases in the literature include involvement of the central nervous system, skin, eye, testis, lung, kidneys, and gastrointestinal tract. In particular, gastrointestinal tract involvement is observed in only 5.1% of cases and affects mostly gastric and colonic sites.^4- 5

The involvement of the gallbladder is rarely seen in any kind of lymphoma and, to our knowledge, our case is the second article in the literature of Richter syndrome with gallbladder localization.⁶

The prognosis of extranodal Richter syndrome including the gastrointestinal localizations is almost invariably very poor and similar to the original form with exclusive nodal presentation. Survival duration is unchanged by aggressive systemic chemotherapy. Furthermore, the role of radiotherapy is only palliative.

Our patient underwent cholecystectomy for palliation and, considering the poor response to both chemotherapy and radiotherapy of the systemic disease, no other adjuvant treatments were administered; no changes in the strategy of cure were developed after surgery and subsequent histological diagnosis of a rare gallbladder localization of Richter syndrome.

Return to Quiz Case.

Correspondence:Marco Pericoli Ridolfini, MD, Catholic University, Department of Oncology, Surgical Oncology Unit, Località Tappino, 86100 Campobasso, Italy (marco.pericoliridolfini@rm.unicatt.it).

Accepted for Publication:November 14, 2008.

Author Contributions:Study concept and design: Caprino. Acquisition of data: Caprino, Ridolfini, Berardi, Sofo, and D’Ugo. Analysis and interpretation of data: Caprino. Drafting of the manuscript: Caprino and Berardi. Critical revision of the manuscript for important intellectual content: Caprino, Ridolfini, Sofo, and D’Ugo. Administrative, technical, and material support: Ridolfini and Sofo. Study supervision: Caprino.

Financial Disclosure:None reported.