Objective:To explore the clinical value of multidisciplinary team (MDT) treatment model for retroperitoneal soft tissue sarcoma (RPS) in the context of a regional medical center.Methods:The diagnosis and treatment of a patient with RPS in MDT model who were admitted to Xiamen Branch, Zhongshan Hospital, Fudan University in October 2020 were summarized, and the literature was reviewed.Results:The patient was a 59-year-old male, he was diagnosed with retroperitoneal INI-1-deficient poorly differentiated sarcoma with multiple systemic metastases. After several rounds of MDT discussions in Shanghai headquarter and Xiamen Branch of Zhongshan Hospital, Fudan University, the patient received systemic antitumor therapy combined with local therapy. After active treatment, the patient obtained a good curative effect. During the follow-up period, the liver and lung lesions slowly enlarged, and the treatment plan was adjusted. The patient had survived for more than 3 years.Conclusions:The diagnosis and treatment of RPS is complex, and the MDT model in the context of a regional medical center can help to formulate the optimal treatment plan for patients and may maximize the survival benefit of patients.

OBJECTIVE: To explore the pathological characteristics and significance of RET proto-oncogene screening in multiple endocrine neoplasia type 2A (MEN2A) with cutaneous lichen amyloidosis (CLA). METHODS: Clinical data of 51 members from 7 unrelated pedigrees of MEN2A-CLA were collected. Systemic clinical investigations including biochemical testing, imaging examination, germline RET variant screening and histopathological examination were carried out. RESULTS: RET gene variants were detected in 28 patients with MEN2A (C634G/F/R/S/W and C611Y) including 12 males and 16 females, with the mean age of diagnosis being (41.1 ± 18.3) years old, which were consistent with their clinical manifestations. The incidence of medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), hyperparathyroidism (HPTH) and CLA among 28 MEN2A patients were 89.3%, 28.6%, 7.1% and 28.6%, respectively. Comparison of the incidence of MTC/PHEO/HPTH and CLA between C611Y and C634G/F/R/S/W, only PHEO and CLA in C611Y were lower than those in C634G/F/R/S/W (P < 0.05; P < 0.05). Among 8 patients with CLA, the male to female ratio was 2 : 6. The clinical features included pruritus in the interscapular region and presence of dry, thickened, scaly, brown pigment, clustered or desquamate-like plaques. The mean onset age of CLA [(18.4 ± 4.6) years] versus the mean age at diagnosis of CLA or MEN2A were significantly different (P < 0.001; P < 0.001). CONCLUSION MEN2A-CLA may be the early clinical manifestation of MEN2A and most frequently occurred along with RET-C634 variant. To facilitate the recognition of MEN2A-CLA, to combine family investigation and screening of RET variant are helpful for early diagnosis and standardized treatment, which can improve the long-term outcome of MEN2A-specific tumors.
Adolescent ; Adrenal Gland Neoplasms ; Adult ; Amyloidosis, Familial ; Carcinoma, Neuroendocrine ; China ; Female ; Humans ; Lichens ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a/genetics* ; Pheochromocytoma ; Proto-Oncogene Proteins c-ret/genetics* ; Skin Diseases, Genetic ; Thyroid Neoplasms/genetics* ; Young Adult

Objective:To evaluate the clinical effect of the treatment of scar formation after severe acne type Ⅳ by acupuncture.Methods:In Henan Traditional Chinese Medicine Hospital, 60 patients with acne who met the inclusion criteria were selected and divided into observation group and control group according to the randomized controlled grouping method. The observation group(9 male and 21 female, age 18-29) was treated with fire needle and salicylic acid.The control group(10 male and 20 female, age 18-27 ) was treated with needle and salicylic acid.The patients in both groups were treated with tanshinone capsule and salicylate acne pill orally.The course of treatment was 8 weeks. The two groups of clinical efficacy, acne severity score scale (Gags) to evaluate symptom improvement, vancouver scar scale, acne scar weight score were observed and compared.Results:Eight weeks after treatment, the effect rate was 90.00% (27/30) in the observation group and 68.97% (20/29) in the control group. The scores of Vancouver scar scale were (3.66 ± 0.91) in the observation group and (5.06 ± 1.09) in the control group ( P< 0.01). The scores of acne scar weight score were (39.40 ± 16.90) in the observation group and (53.16 ± 17.19) in the control group( P< 0.01). The scores of Gags in the observation group and the control group were (10.31 ± 2.14) and (17.55 ± 2.63)( P< 0.01), respectively. Conclusions:The treatment of Ⅳ grade severe acne skin lesions by fire needle is safe, effective, reliable and easy to operate. It can safely and effectively improve the patient’s facial skin lesions, and reduce the possibility of scar formation and pigmentation, so it is worthy of clinical application.

Objective:To evaluate the clinical effect of the treatment of scar formation after severe acne type Ⅳ by acupuncture.Methods:In Henan Traditional Chinese Medicine Hospital, 60 patients with acne who met the inclusion criteria were selected and divided into observation group and control group according to the randomized controlled grouping method. The observation group(9 male and 21 female, age 18-29) was treated with fire needle and salicylic acid.The control group(10 male and 20 female, age 18-27 ) was treated with needle and salicylic acid.The patients in both groups were treated with tanshinone capsule and salicylate acne pill orally.The course of treatment was 8 weeks. The two groups of clinical efficacy, acne severity score scale (Gags) to evaluate symptom improvement, vancouver scar scale, acne scar weight score were observed and compared.Results:Eight weeks after treatment, the effect rate was 90.00% (27/30) in the observation group and 68.97% (20/29) in the control group. The scores of Vancouver scar scale were (3.66 ± 0.91) in the observation group and (5.06 ± 1.09) in the control group ( P< 0.01). The scores of acne scar weight score were (39.40 ± 16.90) in the observation group and (53.16 ± 17.19) in the control group( P< 0.01). The scores of Gags in the observation group and the control group were (10.31 ± 2.14) and (17.55 ± 2.63)( P< 0.01), respectively. Conclusions:The treatment of Ⅳ grade severe acne skin lesions by fire needle is safe, effective, reliable and easy to operate. It can safely and effectively improve the patient’s facial skin lesions, and reduce the possibility of scar formation and pigmentation, so it is worthy of clinical application.

Objective To indentify the cognitive status of Chinese patients to acne and the influencing factors to theirs' cognitive status,so as to provide solid evidences for the prevention and treatment of acne.Methods A self-designed questionnaire was made to conduct this survey of 16,156 acne patients,who seeked to the treatment in the dermatological departments from 112 hospitals in China.The survey consisted of several parts,including the general status of patients,the patients' cognition of occurrence,development and risk factors of acne,whether the first choice was seeking treatment at the hospital when the patients had acne and the condition of selection of skin care products.The factors were analyzed,which could impact the cognition of the patients' behavior of treatment,how did the patients' cognition to influence their medical behavior and skin care as well as the consistency of assessment of the severity of acne by doctors and patients themselves.Results The acne patients studied had the best knowledge of "acne is a skin disease","it not only occurs in the period of adolescence" and "the disease can be prevented and cured",which accordingly accounted for 80.65％,69.16％ and 65.49％ of the total patients respectively.However,the awareness of acne patients to heredity,high sugar and dairy products as risk factors for acne was insufficient,which accounted for 48.72％,42.40％ and 18.25％ of the total patients,respectively.Gender,age,educational level,occupation and health knowledge were the main factors affecting the cognitive level of patients;the survey also found that men,patient with educational level of junior high or even lower educational condition,occupation of labor workers or farmers and patients were lack of health education with poor knowledge of the genetics and dietary were risk factors for acne;patients with age over 36 years or with mild illness had poor knowledge of dietary risk factors for acne;the difference was statistically significant (P＜0.05).The analysis of the influence of cognitive status on medical treatment behavior and skin care showed that the better the cognition,the higher the probability of patients would choose medical treatment as the first choice as well as choosing functional skin care products;the difference was statistically significant (P＜0.05).The consistency of assessment of the severity of acne by doctors and patients was poor (Kappa value ＜0.4),and the assessment of severity of acne by patients was more serious than doctors' assessment.Conclusions Patient's cognitive status will affect their medical behavior and skin care,and there is also a phenomenon that patients have a more serious assessment of their acne condition.It is suggested that health education for acne patients should be strengthened in clinical medicine so as to improve their knowledge of acne as well as preventing from acne effectively.

OBJECTIVETo compare the efficacy and adverse events of adjusted BACOD (bleomycin, doxorubicin, cyclophosphamide, vincristine, dexamethasone) regimen (continuous intravenous infusion) and conventional BACOD regimen (conventional intravenous drip) in the treatment of relapsed and refractory diffuse large B cell lymphoma (DLBCL).

METHODSRetrospective analysis of 63 cases of relapsed or refractory DLBCL patients was performed, 32 patients received conventional BACOD regimen and 31 patients received adjusted BACOD regimen.

RESULTSThe response rates for adjusted group and conventional group were 87.1%(27/31)and 62.5%(20/32), respectively, during a median follow-up of 14(7-84) months. The difference was statistically significant between the two groups (P=0.025). The main adverse events were myelosuppression, gastrointestinal adverse reactions were rarely serious, and there were no serious liver and kidney toxicity. The median overall survival (OS) was 33 months for adjusted group and 12 months for conventional group, there was statistical differences (P=0.019). The median progression free survival (PFS) was 11 months and 8 months for two groups, the difference was not statistically significant (P=0.095). 1-year survival rates were 68.8% for adjusted group and 44.3% for conventional group, there were no statistical differences (P=0.055). The expected 3- and 5-year survival rates of adjusted group were significantly higher than that of conventional group (47.1% vs 12.8%, P=0.002; 37.7% vs 8.5%, P=0.006, respectively).

CONCLUSIONCompared with the conventional BACOD regimen, the adjusted BCOAD regimen is effective and well tolerated in patients with relapsed or refractory DLBCL, the overall response rate and OS increased.

Adult ; Aged ; Aged, 80 and over ; Antineoplastic Combined Chemotherapy Protocols ; Bleomycin ; Cyclophosphamide ; Dexamethasone ; Doxorubicin ; Female ; Humans ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Vincristine

Objective To investigate the clinical characterstics of bone Langerhans-cell histiocytosis (LCH) and evaluate its diagnosis,therapy and prognosis.Methods 25 cases with biopsy confirmed bone LCH during the last 8 years were retrospectively analyzed.Results The patients included 18 males and 7 females,13 children and 12 adults,ranging from 1.5 to 55 years old with a median age of 17.Cases with unifocal lesions were 17,including 11 cases of skull LCH,and the remaining 8 were with multifocal lesions.First symptoms were predominantly pain and local masses,and rarely constitutional symptoms.The manifestation of radiography was osteolytic bony lesions.12 cases had masses in soft tissues.Patients with solitary lesions underwent surgical operation,followed by radiotherapy or chemotherapy.Cases with multifocal lesions received chemotherapy and radiotherapy.Pathological examination showed proliferation of well differentiated histiocytes,and large numbers of infiltrating eosinophils.Positive rates of CD1a,S100,Vimentin and CD68 were higher in immunohistochemistry.Patients with restricted involvement in bones can achieve a satisfactory therapeutic effect.2 cases died when multiple systems were compromised.Conclusion Bone LCH occurs predominantly in children and teenagers,involves solitary bones,and morbidities in males are much higher than females.Skull is most commonly affected.Principal clinical manifestations are pain and local masses.Diagnosis of bone LCH depends on microscopic examination.Combination therapy appears to be an effective method of treatment.Prognosis of disease is related to the degree of bone involvement,histological classification and simultaneously encroachment of other organs.Most patients have good prognosis.

Objective To observe the efficacy and adverse events of L-asparaginasum plus DICE regimen in the treatment of relapsed and refractory non-Hodgkin's lymphoma (NHL). Methods Thirty-one patients with relapsed and refractory NHL were treated with L-asparaginasum plus DICE regimen. Each patient was scheduled to receive 2 to 6 cycles.Results Among the 31 assessable patients,11 (35.5 ％) achieved a complete remission (CR),14(45.2 ％) got a partial remission (PR),2 were stable,4 were progressive.The overall response (CR + PR) rate was 80.7 ％.The median survival was 8 months (rang:2-38 months).The 1-year survival rate was 43.3 ％,the 2-year survival rate was 32.5 ％.The main adverse events were myelosuppression,digestive tract reaction,allergy and edema.No treatment-related death was observed.Conclusion The L-asparaginasum plus DICE regimen is effective and safe for the relapsed and refractory NHL.

Objective To explore the efficacy and safety of moderate-dose of etoposide (VP16) with granulocyte-colony-stimulating factor (G-CSF) for mobilization of peripheral blood stem/progenitor cells.Methods VP16 at 1.2 g/m2 was injected intravenously by six divided doses via a central vein, 2 times every 12 hours for 3 days in 31 patients with malignant lymphoma (30 non-Hodgkin lymphoma and 1 Hodgkin lymphoma). All patients received G-CSF 5 μg/kg were given twice daily subcutaneously from the day of the nadir of white blood cell (WBC) till the day before the last APBSC harvest. Results The mean time for the collection of stem cell was 12 days (10-15) following etoposide chemotherapy. The mean number of mononuclear cell (MNC) and CD+34 cells in collection were 7.8×108/kg (5.2-11.3×108) and 7.2×106/kg (5.3-13.1×106). respectively. 18 patients completed collection with a single apheresis, and 13 patients underwenttwice. All patients were recovered for haematopoiesis in following APBSCT. Median (range) time for the recovery of absolute neutrophil count (ANC)＞0.5×109/L and platelet＞20×109/L were+12 (+9-+18) days and +14 (+10-+21) days respectively. Slight adverse events coursed by the regimen could be tolerated. Conclusion VP16 at moderate dose with G-CSF is an effective and safe mobilizing regimen for autologous peripheral blood stem/progenitor cells in patients with malignant lymphoma. It was suggested to use extensively.

Objective To explore the effective treatment with regimen of remission induction for relapsed/refractory precursor lymphocytic leukemia/lymphoma patients. Methods 6 patients with relapsed/refractory precursor lymphocytic leukemia/lymphoma including 2 acute lymphocytic leukemia, 4 lymphoblast lymphoma and 1 hybrid acute leukemia were treated by combination of CAG regimen with L-asparaginase (L-Asp) and prednisone (PDN). Results All patients responded to the regimen, in which 6/7 (85.7 %)patients achieved complete remission and 1/7 (14.3 %) patient achieved partial remission after one course.Light adverse events coursed by the regimen could be tolerated. Conclusion The regimen consisting of CAG, PDN and L-Asp is worth exploring for relapsed/refractory precursor lymphocytic leukemia/lymphoma.