510655 Guangzhou, China Diao Dechang, Department of Gastrointestinal Surgery, Guangdong Provincial Hospital of Traditional Chinese Medicine published in Chinese Journal of General Surgery [Abstract] Objective To deepen the understanding of focal Castleman disease (LCD) and improve the diagnosis and treatment of LCD. Methods To review and analyze the clinical characteristics and treatment of 26 patients with LCD, and to summarize their clinical characteristics and treatment strategies. The lymph node enlargement was isolated, with a maximum diameter of 1.2-15.0 cm, most often located in the retroperitoneum (10 cases), followed by the mediastinum (7 cases); the imaging examination was specific and helpful for preoperative diagnosis; the histopathological examination was clear vascular in 22 cases and plasma cell in 4 cases. Twenty-five of the 26 patients had complete resection of the tumor and were followed up for 5-206 months, with a mean follow-up of 48.5 months, and only 2 cases had recurrence after surgery. In the other case, only palliative resection was performed because the tumor was deep and adjacent to vital organs, making complete resection difficult. The palliative resected patient and one postoperative recurrence case were given combined chemotherapy regimen for tumor disappearance and no recurrence was seen so far; the other recurrence patient was not given chemotherapy and eventually died. Conclusion LCD mainly presents as isolated enlarged lymph nodes, some patients have systemic symptoms and positive laboratory test results, mastering its imaging characteristics can help preoperative diagnosis, and complete resection of the mass is the most important treatment modality for the disease, which can achieve cure. Diao Dechang, Department of Gastrointestinal Surgery, Guangdong Provincial Hospital of Traditional Chinese Medicine 【Key words】 Castleman’s disease; clinical features; treatment CFL：R735 Document ID：A Clinical features and treatment of localized castleman’s diaease 【 ABSTRACT】 Objective To deepen our understanding of localized Castleman’s disease (Localized Castleman’s disease, LCD). Methods Clinical characteristics and treatment of 26 cases with LCD were Retrospectively analyzed, and its Results Among the 26 cases, there were 10 cases with clinical symptoms, which mainly showed Among the 26 cases, there were 10 cases with clinical symptoms, which mainly showed local pain induced by the compression of the tumors, and 3 in the 10 cases associated with paraneoplastic pemphigus. The swollen lymph node was singly located at a localized area, which was mostly at retroperitoneal (10 cases) and mediastinum (7 cases). The imaging examination of LCD had its special characters and could be helpful for diagnosis. 22 cases were hyaline vascular type, and the other 4 cases The imaging examination of LCD had its special characters and could be helpful for diagnosis. 22 cases were hyaline vascular type, and the other 4 cases were plasma type based on histopathologic examination. 25 of the patients received complete tumor resection and just 2 cases of them recur finally with The duration of follow-up range from 5 to 206 months (the average follow-up time is 48.5 months). One case with the tumor adjoining to some important organs deep in the mediastinum couldn’t achieve complete tumor resection and just This patient after palliative tumor resection and another patient recurred after complete tumor resection both The other patient with recurrent tumor after tumor resection didn’ t receive chemotherapy and died ultimately. Conclusion LCD patients mainly have lymphadenectasis in a single location, and part of them would suffer It is helpful for diagnosis for LCD to be familiar with the feature of the CT scan of LCD. It is helpful for diagnosis for LCD to be familiar with the feature of the CT scan of LCD. Complete surgical resection is the best way to cure this disease. Castleman’s disease (CD), also known as vascular follicular lymphoid hyperplasia or giant lymph node hyperplasia, was described and defined by Castleman in 1956 [1]. Clinically, it is divided into two types, multicentric and focal, depending on the extent of involvement. There are not many large cases of focal CD reported at home and abroad. We now review and analyze the clinical manifestations and diagnosis and treatment of 26 patients with focal CD, and summarize their clinical characteristics and diagnosis and treatment strategies to improve the diagnosis and treatment of focal CD. 1 Data and methods 1.1 General data A total of 31 patients with focal Castleman’s disease (LCD) were admitted to the Guangdong Provincial Hospital of Traditional Chinese Medicine from May 1993 to May 2010, of which we collected 26 patients with well-documented symptoms, signs, ancillary examinations and treatment. Among them, 10 were retroperitoneal, 7 mediastinal, 3 cervical, 2 axillary and 2 pelvic, 1 left upper arm and 1 right inguinal; 14 males and 12 females, age ranged from 4 to 71 years, mean age 39.4 years; follow-up time ranged from 5 to 206 months, mean follow-up time 48.5 months (48.5±13.4), and there were no lost cases. 1.2 Clinical manifestations 1.2.1 Symptoms and signs Among the 10 cases of retroperitoneal LCD, 4 cases showed abdominal pain, 3 cases showed symptoms of paraneoplastic pemphigus (PNP) such as ulceration of the mouth and lips and positive autoantibodies, and the remaining 3 cases were asymptomatic; among the 7 cases of mediastinal LCD, 1 case showed epigastric pain, and the rest were asymptomatic; 2 cases of pelvic LCD showed increased menstrual flow in 2 cases; 1 case of left axillary LCD showed gradually increasing swelling of the left upper limb, and the rest of LCD showed superficial painless swelling. In terms of physical signs, abdominal masses were palpated in 6 LCDs, with sizes ranging from 4.5 cm×4.0 cm to 17.0 cm×14.4 cm; 7 LCDs were located on the body surface and all showed local superficial lymph node enlargement without pressure pain and clear borders, with sizes ranging from 1.2 cm×0.8 cm to 5.5 cm×3 cm; the remaining LCDs had no abnormal physical signs. 1.2.3 Laboratory tests Among the 26 patients with LCD, 6 had elevated urine leukocytes, 5 had proteinuria, 4 had prolonged prothrombin time, 3 had elevated blood leukocytes, 2 had hematuria, and 1 had anemia. 14 had negative HIV antibody, syphilis antibody, and tuberculin test. 1.2.3 Imaging examination Ultrasound, X-ray and CT examination were performed in all 26 patients, and enlarged lymph nodes were found, with tumor diameters ranging from 1.2 cm to 15.0 cm, with an average diameter of 6.7 cm. CT examination of the lesions was characteristic, mainly showing round or round-like masses with irregular margins, individually lobulated, with homogeneous densities, and significant early enhancement and sustained enhancement on delayed scan. The ultrasound showed an isolated round or ovoid hypoechoic mass with uniform internal echogenicity and a well-defined envelope, and abnormal blood flow signals were seen around and inside the mass. It is noteworthy that one of the patients was found to have combined ascending colon cancer at the same time by CT examination. 2. Results 2.1 Histopathological features Twenty-two of the 26 LCD cases were of the hyaline vascular type (as shown in Figure 3,4), and four were of the plasma cell type. The common features of immunohistochemical detection were all polyclonal lymphocytic hyperplasia, positive cells of CD20 and CD79a in the intrafollicular, set and marginal areas, positive expression of follicular dendritic cells in the CD21 germinal center, and scattered positive cells between CD3 follicles. 2.2 Treatment and regression All 26 LCD cases were treated surgically, and 25 cases had complete resection of the mass, and only one case was palliative resection, with a success rate of 96.2%. In the case of palliative resection, the mediastinal LCD was not completely resected because the mass was located between the left pulmonary hilar and the aortic arch, and there were obvious adhesions with the surrounding tissues; the mass subsided after 5 courses of chemotherapy with CHOP regimen and no recurrence was seen on follow-up CT for 38 months. One case of left axillary LCD was resected in ’03, ’05 and ’06, respectively. The pathological diagnosis of the first two surgical resections suggested extraosseous Ewing’s tumor, and the last histopathological and immunohistochemical staining diagnosed plasma cell type CD. No recurrence was found so far. The remaining 23 patients with LCD were followed up postoperatively without recurrence, with the longest follow-up period of 206 months. 3. Discussion 3.1 Clinical characteristics of LCD Focal Castleman’s disease (LCD) has a young age of onset, most of them are asymptomatic, and are often seen for painless enlargement of lymphoid tissue or mass compression symptoms. Testa [3] showed 315 cases of LCD, of which 65% were located in the mediastinum, 16% in the neck, 12% in the abdomen, 3% in the axilla, and 4% in other areas. The most enlarged lymph nodes in our group were located in the retroperitoneum, followed by the mediastinum, which is inconsistent with the literature. The reason for this may be related to the bias in patient admission due to the superiority of our two hospitals in terms of the level of abdominal surgery. Some of the patients in our group showed hematuria, proteinuria, and positive fecal occult blood in laboratory tests, which some scholars believe is likely related to the fact that the patient’s enlarged lymph nodes occurred in the mesenteric root and the enlarged lymph nodes affected the venous return of the spleen and intestinal canal and the blood flow to the liver [4]. All of the cases in our group with abnormal laboratory tests were found to have varying degrees of compression of the surrounding organs by the enlarged lymph nodes. In addition, we found that retroperitoneal LCD was mostly located in the peri-abdominal aortic area of the mid-upper abdomen, which may be related to the distribution pattern of abdominal lymph nodes. There are 2 cases of pelvic LCD in our group, both of which showed increased menstruation and imaging suggestive of pelvic masses, which are easily misdiagnosed as gynecological tumors and should be clinically important. LCD imaging has characteristic features, and preoperative diagnosis is possible as long as its characteristics are mastered. 3.2 Treatment of LCD Surgical complete excision of enlarged lymph nodes or masses is the main and most effective treatment for this disease, which can achieve cure [3]. Once the diagnosis of LCD is made, surgical excision should be prepared as soon as possible, and the swelling should be removed outside the envelope as much as possible during surgery. However, because deep LCD is mostly asymptomatic, the tumor is often large when patients come to the clinic, and the tumor is often close to important organs and has some adhesions with surrounding tissues and organs, making surgical resection difficult, and sometimes difficult to complete. If the first surgical resection is not complete, there is a possibility of recurrence, and those who have recurrence can often undergo surgical resection again. Only 2 of our 25 surgical resections were recurrent, and recurrence may be related to incomplete surgery. In a patient with retroperitoneal LCD, the mass was 15.0 cm in diameter, and the mass was found to have a rich blood supply and obvious adhesions with the surrounding organs during the initial surgery, which made the surgery very difficult and resulted in intraoperative blood loss of 600 ml; 9 years after the surgery, the CT was repeated to suggest recurrence, and the size of the mass reached 13 cm×11 cm, Patients with LCD with deep organ lymph node enlargement that is difficult to remove surgically or recurrence that is difficult to remove radically after elimination can be cured with chemotherapy or radiotherapy according to the lymphoma protocol [5]. In one of our palliative resection cases, chemotherapy with CHOP (cyclophosphamide + vincristine + prednisone) regimen was given after surgery, and the mass disappeared without recurrence at 38 months of follow-up. In addition, in patients with concomitant PNP or systemic symptoms, preoperative application of sensitive antibiotics is required to control infection and control skin damage as well as systemic symptoms [6]. Large skin lesions and more pronounced systemic symptoms often suggest high titers of autoantibodies in patients, and high doses of gammaglobulin should be given intraoperatively to neutralize autoantibodies, and surgical operations should be performed gently without squeezing the tumor to avoid massive release of antibodies produced by tumor cells, which may lead to a transient increase in postoperative fever and dyspnea. We have a patient with retroperitoneal LCD with PNP who developed severe postoperative systemic inflammatory response syndrome due to difficult separation and significant extrusion of the tumor body during surgery. Fig. 1 CT plain scan: large retroperitoneal soft tissue mass with smooth margins and uniform density. Fig. 1 CT plain scan showed a large round-like well-defined mass, with homogeneous density. Fig. 2 Enhanced CT scan: the lesion was more clearly enhanced, with clear demarcation from surrounding tissues, and the surrounding tissue structure was clearly nudged. Fig. 2 Enhanced CT scan showed the lesion was apparently strengthened with a clear limit out of the surrounding tissue. Fig. 3 Clear-vessel Castleman disease: disappearance of the lymphatic follicle-generating center. The interfollicular capillaries were proliferated in large numbers and had an onion skin-like appearance (HE,×100). Fig. 3 Pathologic photography of a hyaline-vascular Castleman’s disease under High-powered Microscope: An onion skin-like look, showing lymphoid follicle germinal centers disappeared and the capillaries between the follicles were highly hyperplasia with a huge number.(HE× 100) Fig. 4 Plasma cell type Castleman disease. A large number of plasma cells can be seen in the interfollicular area, and vascular hyperplasia and onion skin-like changes are not obvious. (HE × 400) Fig. 4 Pathologic photography of a Plasma cell type Castleman’s disease under High-powered Microscope: A large number of plasma A large number of plasma cells infiltrate the interfollicular areas, while the change of angiogenesis and onion-like is not obvious. (HE × 400) References [1] Herrada J, Cabanillas F. Rice L, et al. The clinical behavior of localized and multicentric Castleman disease.[J]. Ann Intern Med,1998,128(8):657-662. [2] Liu N, Qiu F B, Li F D, et al. 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