Updated retinoblastoma incidence and outcome in children in Taiwan from 1980 to 2019: a 40-year nationwide study.

BACKGROUND: Retinoblastoma is a potentially fatal disease, and its incidence and mortality varies among different countries and periods. METHODS: This is a nationwide population-based retrospective study from January 1980 to December 2019 in Taiwan. Patients diagnosed as retinoblastoma were identified from the Taiwan National Cancer Registry. To update the literature on retinoblastoma incidence, mortality and trends in Taiwan, we analysed changes in incidence and survival rates over time according to sex, diagnostic age, laterality and treatment. RESULTS: During 1980-2019, the incidence of retinoblastoma in Taiwan was 1 per 16 489 live births (95% CI: 13 415-19 564). The diagnostic age decreased from 2.21 ± 0.26 during 1980-1984 to 1.24 ± 0.26 during 1985-2019. Compared with that observed during 1980-1989, the incidence rate observed after 1990 increased significantly in children aged <10 years (RR: 1.62-2.40, P = 0.0049 to < 0.0001). From 1980 to 2019, the incidence rate for the 0-4-year age group increased and that for the 5-9-year age group remained constant. The mean diagnostic age for bilateral retinoblastoma (0.36 ± 0.47 years) was significantly less than that for unilateral retinoblastoma (1.37 ± 0.35 years) during 2007-2019 (P < 0.0001). The 10-year survival rate was highest in the enucleation group (89.8%) compared with radiotherapy (52.2%) and others (70.0%; P < 0.0001). CONCLUSIONS: During 1980-2019 in Taiwan, the incidence of retinoblastoma increased significantly, and the diagnostic age decreased, which are similar to the ones from other developed countries. However, the survival rate was still lower than that of most developed countries.

Novel chlorin e6-based conjugates of tyrosine kinase inhibitors: Synthesis and photobiological evaluation as potent photosensitizers for photodynamic therapy.

Since tyrosine kinase inhibitor (TKI) could reverse ABCG2-mediated drug-resistance, novel chlorin e6-based conjugates of Dasatinib and Imatinib as photosensitizer (PS) were designed and synthesized. The results demonstrated that conjugate 10b showed strongest phototoxicity against HepG2 and B16-F10 cells, which was more phototoxic than chlorin e6 and Talaporfin. It could reduce efflux of intracellular PS by inhibiting ABCG2 in HepG2 cells, and localize in mitochondria, lysosomes, golgi and ER, resulting in higher cell apoptosis rate and ROS production than Talaporfin. Moreover, it could induce cell autophagy and block cell cycle in S phase, and significantly inhibit tumor growth and prolong survival time on BALB/c nude mice bearing HepG2 xenograft tumor to a greater extent than chlorin e6. Consequently, compound 10b could be applied as a promising candidate PS due to its good water-solubility and stability, low drug-resistance, high quantum yield of 1O2 and excellent antitumor efficacy in vitro and in vivo.

[Clinicopathologic features of micropapillary variant of pure mucinous carcinoma of breast].

OBJECTIVE: To study the clinicopathologic features of pure mucinous carcinomas of the breast with diffuse micropapillary pattern. METHODS: Twenty-six cases of micropapillary variant of pure mucinous carcinoma of the breast were retrospectively reviewed by light microscopy, immunohistochemistry and clinical data analyses. RESULTS: The age of 26 female patients ranged from 30 to 77 years old, of which 12 cases with clinical details available were mean 54 years old. The tumor diameter ranged from 0.8 to 9.0 cm (mean 3.2 cm). Ipsilateral axillary nodal metastases were identified in 3 cases. Cutaneous involvement was also found in 2 cases. The tumor cells showed the similar architectural arrangement as in invasive micropapillary carcinoma, with peripheral borders of the cell clusters highlighted by epithelial membrane antigen. Various amount of mucin occupied the retraction spaces around the tumor cells. Compared with conventional pure mucinous carcinoma of the breast, mucinous carcinomas with micropapillary pattern showed different nuclear grades (19 cases of grade I, 2 cases of grade II, 5 cases of grade III). The micropapillary cell clusters varied in size (22 cases of big micropapillary and 4 cases of small). Intraductal carcinoma was observed in 12 cases. Calcification and psammoma bodies were observed in 8 cases. Immunophenotyping, the tumor cells were with higher expression of hormone receptors, but HER2 were negative. Ki-67 positive index was 1% ∼ 70%. Neuroendocrine differentiation was observed in 6 cases. CONCLUSIONS: The micropapillary variant of pure mucinous carcinoma of the breast, which mainly occurs in younger women, may carry the similar propensity for angioinvasion and nodal metastasis as infiltrating micropapillary carcinoma at least in cases with high nuclear grade. This morphologic subtype needs to be distinguished from conventional pure mucinous carcinoma of the breast and treated properly.

Primary central nervous system plasmablastic lymphoma presenting in human immunodeficiency virus-negative but Epstein-Barr virus-positive patient: a case report.

We report a 32-year-old Outer Mongolian man, with plasmablastic lymphoma (PBL) primarily occured in the central nervous system and diagnosed by surgical resection. This patient appeared headache and magnetic resonance imaging (MRI) showed multiple lesions in the right cerebral hemisphere including the right frontal-parietal lobe and right basal ganglia and the left cerebellum, he was diagnosed as lymphoma by stereotactic biopsy in January 2009 in local hospital, and was given radiotherapy 33 times after the biopsy. The patient was admitted to The Military General Hospital of Beijing PLA., Beijing, P.R. China on March 9th, 2011, with chief complaints of right limbs convulsioned suddenly, then fell down and lose of his consciousness, then awoke after 4 to 5 minutes, with symptoms of angulus oris numbness and the right upper limb powerless ten days ago.MRI of the brain revealed a well-defined hyperdense and enhancing mass in the left frontal-parietal lobe, the meninges are closely related, there was extensive peritumoural edema noted with pressure effects, as evident by effacement of the left lateral ventricles and a 0.5 cm shift of the midline to the right side.Surgical resection showed markedly atypical, large singly dispersed or cohesive proliferation of plasmacytoid cells with frequent abnormal mitoses and binucleation, some neoplastic cells were large with round or oval nuclei and showed coarse chromatin and smaller or unapparent nucleoli, some neoplastic cells with prominent nucleoli, apoptosis and necrosis were often presented. Immunohistochemistry staining and gene rearrangement together with other supportive investigation confirmed the diagnosis of primary central nervous system plasmablastic lymphoma. A month later, he was started on chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, leurocristime and prednisone) for a week. Other supportive treatment was provided for symptomatic epilepsy. The patient regained muscle strength in both upper limbs and right lower limb and the symptomatic epilepsy was controlled after two weeks. Then the patient was discharged. Follow-up data shows the patient to be alive eleven months after discharge. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1649317674697046.

[Neuroendocrine carcinoma of breast: a study of tumor morphology and subtyping].

OBJECTIVE: To study the clinical features and histopathology of the neuroendocrine carcinoma (NEC) of the breast. METHODS: Twenty-two cases of NEC of the breast were analysed by morphology and immunohistochemistry using synaptophysin, chromogranin A, NSE, CD56, estrogen receptor (ER), progesterone receptor (PR), HER2, EGFR, CK5/6, CK14, p63, E-cadherin, p120, p53 and Ki-67 staining. HER2 gene amplification was detected by fluorescence in situ hybridization (FISH) for cases with HER2 protein expression 2+. The diagnosis of breast NEC relies on the expression of neuroendocrine markers expression in more than 50% of tumor cells, and no evidence of neuroendocrine carcinoma in any other parts of the body at the same time. RESULTS: The patients aged from 31 to 96 years (mean 65.2 years), and all were female but one. Amongst the 22 patients studied, the NECs were in the left breast in 15 cases (68.2%) and in the right breast in seven cases (31.8%); the tumor size was 0.5 to 5.5 cm (mean 2.7 cm). Lymph node metastasis was found in six cases. Basing on the morphologic features, these 22 cases were categorized into six subtypes including nine cases of solid cohesive, six of mucinous, three of solid papillary, two of small cell, one of large cell and one of alveolar variants. Immunohistochemically, the expression rate of markers was 100% (22/22) for synaptophysin, 12/13 for NSE, 54.5% (12/22) for chromogranin A, and 5/16 for CD56. Also, 90.5% (19 of 21) of cases expressed ER, 81.0% (17 of 21) of cases expressed PR, and none expressed EGFR, CK5/6, CK14 and p63. HER2 protein over-expression (3+) and gene amplification was not detected in any case. All cases (19/19) were positive for membrane staining for E-cadherin and p120. p53 expression was seen in 6 of 17 cases. Ki-67 labeling index was less than 3% in 9.5% (2/21) of the cases, 3% to 20% in 66.7% (14/21) of the cases and more than 20% in 23.8% (5/21) of the cases. Both cases of HER2 (2+) did not show gene amplification by FISH. On the basis of immunophenotypes, most of the breast NECs were of the luminal molecular subtype, but not HER2-overexpression or basal-like subtypes. CONCLUSIONS: NEC of breast more likely occurs in elderly patients and in the left breast than the right breast. The most common morphology is the solid cohesive subtype, followed by the mucinous variant.