Diagnosis and treatment of pleomorphic rhabdomyosarcoma of the uterus: a rare case report and review of the literature.

Pleomorphic rhabdomyosarcomas of the uterus (PRMSu) is a rare malignant tumor of the female genital tract. Accurate diagnosis and effective treatment of PRMSu are important. We report an 81-year-old woman who was diagnosed with PRMSu. She had an extremely unusual presentation of secondary dyspnea because of an extremely large uterus (26.0 cm). Pelvic magnetic resonance imaging showed rare severe enlargement and intrauterine filling with tumor tissue, and she was initially diagnosed with uterine leiomyosarcoma. The patient underwent hysterectomy, as well as bilateral salpingo-oophorectomy and omentectomy, and was finally confirmed as having PRMSu by histopathology combined with immunohistochemistry. We performed a systematic review of the literature between 1982 and 2020 and focused on different treatment strategies and prognosis of PRMSu. A retrospective review of 28 cases was conducted and survival analysis was estimated by using the Kaplan-Meier method. We found that the accuracy of diagnosis of PRMSu completely depends on histopathology and immunohistochemistry because of no special clinical symptoms, no sensitive tumor markers, and no special imaging findings. Although there is no standardized approach for treating this rare disease, the treatment strategy of a surgical operation combined with adjuvant chemotherapy appears to be the best choice.

[Establishment of reference ranges of sex hormones for healthy children in Shenzhen, China based on chemiluminescence].

OBJECTIVE: To study the reference ranges of six sex hormones, i.e., luteinizing hormone, follicle-stimulating hormone, progesterone, prolactin, estradiol, and testosterone, for healthy children aged 0-18 years in Shenzhen, China. METHODS: Stratified cluster sampling was performed to select 2 178 healthy children aged 0-18 years in the districts of Futian, Luohu, Nanshan, Bao'an, and Longgang in Shenzhen between September 2015 and September 2016. There were 1 219 boys and 959 girls, including 81 neonates, 335 infants, 346 young children, 469 preschool children, 419 school-aged children, and 528 adolescents. The American Beckman DXI800 chemiluminescence meter was used to measure the levels of luteinizing hormone, follicle-stimulating hormone, progesterone, prolactin, estradiol, and testosterone. RESULTS: There were significant differences in the levels of luteinizing hormone, follicle-stimulating hormone, progesterone, prolactin, estradiol, and testosterone between different age groups (P<0.05). There were also significant differences in the levels of these sex hormones between boys and girls in the same age group (P<0.05). The reference ranges of six sex hormones were established for healthy children aged 0-18 years in Shenzhen based on the levels of these hormones in different age groups. CONCLUSIONS: There are significant differences in sex hormones between different age groups or sex groups. The reference ranges of six sex hormones established for different sexes or ages have great significance in the diagnosis and treatment of endocrine diseases in children.

[IL-12-induced expression of TRAIL enhances the cytotoxicity of NK cells against Jurkat cells].

AIM: To study the mechanism underlying the IL-12-induced cytotoxic function of NK cells to Jurkat cells. METHODS: NK cells from peripheral blood mononuclear cells (PBMCs) were purified by magnetic sorting and stimulated with or without IL-12. The expression of genes on IL-12-treated and non-IL-12-treated NK cells was analyzed by gene chips and the expression of cytolytic molecules was evaluated by flow cytometry. RESULTS: Seventeen genes were up- (5/17) or down-regulated (12/17) on IL-12-treated NK cells compared with non-IL-12-treated NK cells (fold change≥10). IL-12-induced expression of TRAIL on NK cells mediated the cytotoxicity to Jurkat cells. The expression of TRAIL on subsets of CD56(+);CD16(+); and CD56(-);CD16(+); NK cells significantly increased after the stimulation with IL-12 and Jurkat cells expressed high level of TRAIL receptor 2 (TRAIL-R2). Importantly, the neutralizing mAbs against TRAIL (RIK-2) significantly inhibited the cytotoxicity of NK cells induced by IL-12. CONCLUSION: The expression of TRAIL on human NK cells induced by IL-12 was one of the major mechanisms of cytotoxicity to Jurkat cells.

[Effect of different dietary loads on glucose-dependent insulinotropic polypeptide in subjects with normal glucose tolerance].

OBJECTIVE: To investigate the secretion patterns of glucose-dependent insulinotropic polypeptide (GIP) after different dietary loads in subjects with normal glucose tolerance (NGT) and their relation to insulin secretion and plasma glucose levels. METHODS: Fourteen subjects with normal glucose tolerance underwent 75 g glucose tolerance test(OGTT) followed by mixed meal tolerance test(MMT) one week later. Blood glucose, insulin, and GIP were measured in the fasting state and at 0, 15, 30, 60, 90 and 120 min after glucose load or mixed meal load. RESULTS: The first peak value of GIP after glucose load occurred at 15 min (45.09∓4.67 pmol/L). After a brief decline, GIP continued to increase till reaching 59.66∓11.73 pmol/L at 120 min after the load. After the mixed meal load, GIP secretion presented with two peaks: the first peak appeared at 15 min (71.69∓14.19 pmol/L) with a level significantly higher than that at 15 min following glucose load (P<0.05), and the second occurred at 90 min (55.35∓13.19 pmol/L). The area under curve of GIP showed no significant difference between the two loads (P>0.05). Compared with glucose load, mixed meal load resulted in an increase of the first GIP peak and an earlier insulin peak (30 min vs 60 min), but a significant decrease of blood glucose at 15 min (P<0.05). CONCLUSION: Compared with glucose load, mixed meal (containing fat) can strongly stimulate GIP release and cause earlier occurrence of the insulin peak, which might be an important reason for the lower blood glucose after mixed meal.

Proposed classification of lens capsule defects.

BACKGROUND: Capsule defects are common during or after intraocular surgery of various kinds. The purpose of this work is to establish a classification system of lens capsule defects to provide uniform description of these defects for ophthalmic research and IOL implantation. METHODS: A retrospective study of 128 patients (156 eyes) with lens capsule injury after ocular trauma and intraocular surgery was performed. The patients were divided into two groups. Capsule defects were defined and classified according to the location, size, shape and tension of the capsule and its effect on posterior chamber IOL implantation. RESULTS: Lens capsule defects were classified into four types: Type I - complete capsule; Type II - incomplete capsule, but has enough area and tension to support two IOL haptics; Type III - incomplete capsule, is able to support only one IOL haptic and the other haptic needs a suture; Type IV - no capsule, both IOL haptics need suture fixation. Type I and Type II were each divided into three subtypes. Type III was divided into two subtypes. The shape of the capsule defects included fissure-like, triangle, round, irregular and fan-like. All eyes with capsule defects can be sorted into one of these types, and it is easy to guide IOL implantation according to the classification. Type II was the most common among the two groups in this study. CONCLUSION: The classification of lens capsule defects is feasible and favorable for uniform clinical description, clinical research and IOL implantation.

[Clinical study on combination of acupuncture, cupping and medicine for treatment of fibromyalgia syndrome].

OBJECTIVE: To observe the therapeutic effect of acupuncture at five mental points and moving cupping on the Hechelu of the back on fibromyalgia syndrome (FS). METHODS: Sixty-six cases who conformed to the criteria were randomly divided into the treatment group treated with acupuncture at five mental points, moving cupping on the Hechelu of the back and amitriptyline, and the control group treated with amitriptyline. Clinical therapeutic effects were assessed with McGill Pain Questionnaire (MPQ) and HAMD depression scale. RESULTS: The therapeutic effect of the treatment group was better than that of the control group with a significant difference between the two groups (P < 0.01). CONCLUSION: Combination of acupuncture with cupping therapy is an effective therapy for fibromyalgia syndrome.

[Cancer of the nasal cavity].

OBJECTIVE: To analyze the factors affecting prognosis of patients with nasal carcinoma. METHODS: 163 patients treated from 1985 to 1998 were analyzed. The survival analysis was performed by Kaplan-Meier estimate and the comparison between groups by Log-rank test. Multivariate analysis was carried out by Cox proportional hazard model. RESULTS: The overall 5-year survival rate was 58.2%. The 5-year survival rate was 55.8% in squamous-cell carcinoma, 44.0% in adenocarcinoma, 59.7% in undifferentiated carcinoma, 76.3% in adenoid cystic carcinoma, 71.4% in mucoepidermoid carcinoma, 25.0% in rhabdomyosarcoma, 26.7% in malignant melanoma, 50.0% in neuroblastoma (P > 0.05). Patients with cervical metastasis gave a 5-year survival of 53.5% while those without gave 58.9% (P > 0.05). Patients with involvement of sphenoidal sinus or maxillary sinus gave the worse survival. The 5-year survival rate was 73.8% in patients whose cancer completely disappeared after treatment. It was 41.6% in patients whose cancer incompletely disappeared, and 34.3% in patients whose cancer remained refractory (P < 0.01). The 5-year survival was 78.3% in stage I disease, 56.4% in stage II disease, 54.2% in stage III and 35.9% in stage IV (P < 0.05). The 5-year survival rate of patients who were treated with radiotherapy only was 56.9%. That of patients who were treated with surgery only was 56.6%. That with chemotherapy only was 25.0% whereas that of patients treated with combination treatment was 61.8% (P > 0.05). So far, 85 patients have died up to writing this report, 57.6% (49 patients) of recurrence or uncontrolled. CONCLUSION: Clinical stage, immediate therapeutic response and involvement of sphenoidal or maxillary sinus; but not the pathologic type, the presence of cervical metastasis nor the method of treatment, are the factors affecting the prognosis of patients with nasal carcinoma. Recurrence and uncontrolled disease are the cause of death.

[Clinicopathological characteristics of atypical cystic duct (ACD) of the breast: assessment of ACD as a precancerous lesion].

OBJECTIVE: To assess the clinicopathological features of atypical cystic duct (ACD) as a precancerous lesion of the breast. METHODS: Whole mammary gland serial sections were performed on 200 cases of breast cancer without pre-operative biopsy (prior operation, fine needle aspiration or needle biopsy were routinely performed in each case). The clinicopathological findings and immunohistochemical features of ACD were investigated. RESULTS: Forty-four (22%) of the 200 breast cancer patients had ACD breast lesions. The frequency of patients with ACD increased in premenopausal women (P=0.001). A number of ACD lesions displayed a histological transition to adjacent ductal carcinoma in-situ. In 16 of 44 (36%) patients with ACD, carcinoma cells stained positive for p53. In 12 of these 16 cases (75%), ACD cells also stained positive for p53 protein (P=0.001). Myoepithelial cells of ACD appeared attenuated and stained strongly for alpha-smooth muscle actin. There was no correlation between the ACD-present group and the ACD-absent group in tumor size, nodal metastasis, and immunostaining patterns of estrogen receptor (ER), progesterone receptor (PR), p53, c-erbB-2 and Ki-67 labeling index of cancerous tissues. All 44 ACD lesions showed a negative staining of c-erbB-2, regardless of the staining result in their corresponding carcinomas. The mean Ki-67 labeling index of ACD lesions was low. CONCLUSIONS: ACD is frequently associated with breast cancer. It may represent a precancerous mammary lesion, supported by the frequent histological continuum between ACD and malignancy, and simultaneous p53 over-expression present in both ACD and its corresponding breast carcinoma.

[Relationship between biologic behavior and morphologic features of invasive micropapillary carcinoma of the breast].

OBJECTIVE: To clarify the relationship between biologic behavior and morphologic features of invasive micropapillary carcinoma (IMPC) of the breast. METHODS: Two thousand and eighty-eight cases of clinically defined monocentric breast cancer without pre-operative biopsy (except fine needle aspiration procedure) were examined by whole mammary gland serial sectioning. The clinicopathologic and morphologic features (including microscopic and ultrastructural) of IMPC were analyzed. RESULTS: One hundred and seventeen cases of IMPC (6.2%, 117/1 880) were diagnosed during the period of study. The incidence of lymphovascular invasion (54.7%, 58/106) and nodal metastases (76.4%, 81/106) was significantly higher in IMPC, as well as the number of metastatic node (on average 9.6) was significantly more in IMPC, as compared with that of the invasive ductal carcinoma. Microscopically, the tumor was characterized by morula-like clusters and small papillae of malignant cells floating within irregular interstitial spaces and separated by fibrous septa. Ultrastructurally, microvilli were observed on the neoplastic cell surface at the periphery of the micropapillae. There were also numerous fine intermediate filaments in the cytoplasm. Newly formed capillaries were noted in the interstitium and some tumor cells were directly in contact with endothelial cells. CONCLUSIONS: A predominant component of IMPC in breast carcinoma is associated with a higher risk of lymphovascular invasion and nodal metastasis. The aggressive behavior of IMPC can be attributed to the proliferative activity of the tumor cells, and its associated angiogenesis.