Survival and ocular preservation in a long-term cohort of Japanese patients with retinoblastoma.

BACKGROUND: Retinoblastoma is an ocular tumor in infants with cancer predisposition. Treatment of the rare tumor needs to be optimized for ocular preserved survival without second primary malignancy (SPM). METHODS: We studied the outcomes of all patients with retinoblastoma at a tertiary center in 1984-2016, when preservation method changed from radiotherapy (1984-2001) to systemic chemotherapy (2002-2016). RESULTS: One-hundred sixteen infants developed unilateral- (n = 77), bilateral- (n = 38), or trilateral-onset (n = 1) tumor. Ten (8.6%) had a positive family history, despite a few studies on RB1 gene. Contralateral disease occurred in one unilateral-onset case. One-hundred eight of 155 eyes (70%) were enucleated. Nine binocular survivors were from 5 bilateral- and 4 unilateral-onset cases. Two survivors received bilateral enucleation. Six deaths occurred; brain involvement (including 3 trilateral diseases) in 4 bilateral-onset, systemic invasion in a unilateral-onset, and SPM (osteosarcoma) in a bilateral-onset case(s). Two others survived SPM of osteosarcoma or lymphoma. The 10-year overall survival (OS: 98.5% vs. 91.3%, p = 0.068) and binocular survivors (13.2% vs. 5.2%, p = 0.154) between bilateral- and unilateral-onsets did not differ statistically. The 10-year OS and cancer (retinoblastoma/SPM)-free survival (CFS) rates of all patients were 94.9 and 88.5%, respectively. The proportion of preserved eyes did not differ between radiotherapy and chemotherapy eras. The CFS rate of bilateral-onset cases in systemic chemotherapy era was higher than that in radiotherapy era (p = 0.042). The CFS rates of bilateral-onset patients with neoadjuvant chemotherapy (upfront systemic therapy for preservation) was higher than those without it (p = 0.030). CONCLUSIONS: Systemic chemotherapy and local therapy raised OS and binocular survival rates of bilateral-onset patients similarly to those of unilateral-onset patients. All but one death was associated with a probable germline defect of the RB1 gene. Neoadjuvant stratified chemotherapy may support the long-term binocular life with minimized risk of SPM.

Clinical features and prognosis of conjunctival melanoma in Japanese patients.

PURPOSE: To evaluate the clinical features and prognosis of conjunctival melanoma in Japanese patients. STUDY DESIGN: Retrospective observational case series. METHODS: Twenty patients (8 men and 12 women) diagnosed with conjunctival melanoma at a singlehospital between 2003 and 2017 were analyzed. Data on clinical presentation, sex, age, the affected eye, tumor location, tumor origin, tumor stage according to the American Joint Committee on Cancer staging system (eighth edition), treatment, outcomes, local recurrence, metastasis, and survival were extracted from the patients' medical records and reviewed. RESULTS: The mean age at diagnosis was 64.2 ± 14.8 years. Tumor locations at the first examination included the bulbar conjunctiva (n = 19), plica (n = 13), and fornix (n = 12). The tumor stage was T1 in 5 cases (25%), T2 in 12 cases (60%), T3 in 3 cases (15%), and T4 in none. The mean follow-up duration was 91.7 ± 46.0 months. The local recurrence rates at 1, 5, and 10 years were 5.0%, 18.8%, and 31.5%, respectively, whilst the metastasis rates were 5.0%, 25.6%, and 32.4%, respectively. Four of the 6 patients who experienced metastasis died; duration from metastasis to death was 17.5 months (range, 7-25). The 5-year survival rate for conjunctival melanoma was 78.8%. Tumor thickness was significantly associated with survival duration on univariate Cox regression analyses. CONCLUSION: The mortality rate for conjunctival melanoma in the Japanese population was lower and higher than that reported in the Chinese and United States populations, respectively. Tumor thickness was a prognostic factor for survival in patients with conjunctival melanoma.

Two Cases of Angiosarcoma with Persistent Unilateral Eyelid Swelling.

Angiosarcoma is a widely known neoplasm with one of the poorest prognoses; however, such cases are rarely observed by the ophthalmologists in clinical practice. The tumor commonly develops on the face and scalp and may cause eyelid swelling. We present 2 such cases. Case 1 was an 82-year-old woman who presented with indulated swelling of the right eyelid that extended to the upper forehead. Biopsy revealed a poorly circumscribed tumor infiltrated throughout the dermis and fat tissue, exhibiting both angiomatous and solid patterns. Immunohistochemistry showed CD31(-) and D2-40/podoplanin(+), suggesting angiosarcoma. The patient had multiple lung metastases and died 14 months later. Case 2 was a 77-year-old man who presented with swelling of the left eyelid, and erythema on the left temporal skin developed ulcerated nodules. Biopsy showed proliferated tumor cells in a sheet form, extensively throughout the dermis. Angiosarcoma was preferred based on immunohistochemistry: CD31(+), CD34(+), and D2-40(-). The patient was treated with electron beam therapy and chemotherapy and died after 13 months. It would be difficult for an ophthalmologist to suspect such a rare neoplasm only from swelling of the eyelids. However, angiosarcoma should be considered if the swelling spreads steadily from a head rash with a nodule or ulcer nearby.

A case of pancreatic neuroendocrine tumors.

Pancreatic neuroendocrine tumors (pNETs) are an uncommon malignancy arising from the neuroendocrine cells of pancreas. Most cases of pNETs present with metastatic disease, but there are few reports in the literature describing pNETs metastasis to the lung and mediastinal lymph nodes [1]. Moreover, although a multimodal treatment including surgical resection and chemotherapy is acceptable for management of pNETs, advanced pNETs still remain a difficult therapeutic challenge [2, 3]. Radiotherapy or combined chemoradiotherapy has not been standard in the treatment of pNETs. An 80-year-old female was admitted to our hospital with cough and anorexia. She had been diagnosed and resected pNETs 8 years ago. Mass shadow was pointed out with chest X-ray, and endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) was performed. Pathological examination revealed neuroendocrine tumors, so the lung mass was considered as metastasis of pNETs. Then, we discussed her treatment at Cancer Board, and radiotherapy was chosen. We hope this case suggests that radiotherapy will be one of the treatment options for metastatic pNETs.