We reviewed the clinicopathological characteristics of 14 patients who underwent resection of basaloid squamous carcinoma (BSC) of the esophagus. The mean age was 65.3 years, and all patients were male. Seven patients had superfi cial BSCs and 7 had advanced BSCs. BSCs were associated with high rates of lymph node metastases and venous invasion. With regard to immunohistochemistry, the rate of cyclin D1 expression was higher (13/14), and the preservation rate of E-cadherin expression was lower (4/14), than that seen in ordinary esophageal squamous cell carcinoma. Squamous cell carcinoma components were most often found at sites of lymph node metastases, whereas basaloid components predominated at sites of hematogenous metastases in 4 patients, including lung, brain, and liver. Seven of the 9 patients with stage I or II disease are alive without recurrence. All patients with stage III or IV tumors developed recurrent disease. Primary tumors responded well in 3 patients who received preoperative chemotherapy (5-fl uorouracil plus cisplatin) and/or radiotherapy, but further studies are needed to clarify the role of chemoradiotherapy. We believe that control of the hematogenous spread of basaloid components may lead to improved outcomes in patients with esophageal BSC.

Fanconi anemia is a congenital syndrome characterized by hypoplasia of bone marrow and the development of aplastic anemia in childhood, followed by myelodysplastic syndrome and acute myelogenous leukemia in later life. We report here a patient first diagnosed with Fanconi anemia at age 10. Bone marrow transplantation was performed at age 23 and repeated after an episode of rejection at age 25. Hematologic findings returned to normal, but chronic graft-versus-host disease persisted. Esophageal cancer developed at age 35. Invasion of the bronchus and aorta by the tumor was suspected on computed tomography. Chemoradiotherapy was administered to down-stage the tumor, using low-dose cisplatin and 5-fluorouracil. After two courses of chemotherapy with cisplatin (total dose, 100 mg) and 5-fluorouracil (5000 mg) plus radiotherapy (30 Gy), Grade 3 diarrhea and bone marrow suppression developed, and treatment was discontinued. After resolution of toxicity, a good response to the neoadjuvant therapy was seen on computed tomography scan, and a subtotal esophagectomy was performed which demonstrated a complete response in the resected specimen. However, tongue cancer developed at age 40 years, and hemiglossectomy was performed. Patients with Fanconi anemia have a high risk of developing esophageal cancer while they are still young. Reduced doses of alkylating agents and radiotherapy are used in patients with Fanconi anemia. However, the optimal dosage of chemoradiotherapy and the treatment strategy for esophageal cancer in patients with Fanconi anemia remain unclear, and outcomes are generally extremely poor. In this patient, esophageal cancer associated with Fanconi anemia responded well to multidisciplinary therapy.

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To prevent ovarian dysfunction due to total body irradiation, we started ovarian shielding at our center (Saitama Medical Center, Jichi Medical University (SMC-JMU)) with a long source axis distance, which is different from the original method used at the University of Tokyo Hospital (UTH). We retrospectively analyzed the outcome of eight patients with a median age of 20.5 years from SMC-JMU and compared the results with the published data for eight patients with a median age of 22 years from UTH. The recovery of ovarian function was observed in five and six patients, respectively. The cumulative incidence of ovarian recovery, while treating relapse and death without ovarian recovery as competing risks, was 68.8 % at 2 years after transplantation in the total population, and there was no statistically significant difference between the two institutions (p = 0.85). Age and the history of previous chemotherapy did not affect the incidence of ovarian recovery. Two patients from each center had a relapse of leukemia. Overall, among the 11 patients who have survived without relapse, only one has not achieved ovarian recovery. In conclusion, ovarian shielding with both methods strongly protected ovarian function. However, we should continue to monitor the relapse rate among patients who undergo this procedure.