Neurocutaneous melanosis is a rare congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic dilatation of the fourth ventricle and agenesis or hypoplasia of the cerebellar vermis. We studied a case of Dandy-Walker syndrome with neurocutaneous giant melanosis in a 3 years old boy.
Brain ; Child, Preschool ; Dandy-Walker Syndrome* ; Dilatation ; Fourth Ventricle ; Humans ; Male ; Melanosis* ; Neurocutaneous Syndromes ; Nevus ; Parturition ; Seizures ; Skin

PURPOSE: In breast cancer, response to endocrine therapy depends on estrogen receptor and progesterone receptor status. However, poor prognosis is conferred on patients with hormone receptor (HR)-positive breast cancer. We aimed to examine weakly positive HR breast cancer by comparing weakly positive HR to strongly positive HR and negative HR breast cancer. METHODS: We examined the clinical and biological features of 1,496 women with breast cancer, and these patients were categorized according to HR status as weakly positive, strongly positive, and negative HR breast cancer. RESULTS: In this study, among 1,496 patients with breast cancer, negative HR breast cancer was found in 374, weakly positive HR breast cancer in 90 and strongly positive HR breast cancer in 1,032 patients. Our multivariate analysis showed that there were differences in T stage, tumor-node-metastasis stage, vascular invasion, histologic grade and type, and Ki-67 index. Patients with weakly positive HR breast cancer had an increased risk of death and recurrence compared with those with strongly positive HR breast cancer and had similar prognosis as patients with negative HR breast cancer. CONCLUSION: Patients with weakly positive HR breast cancer received endocrine therapy because they were regarded as having positive HR breast cancer. However, their prognosis of overall survival and relapse-free survival was similar to that in patients with negative HR breast cancer. Therefore, we need to closely observe and consider active treatment for patients with weakly positive breast cancer.
Breast Neoplasms ; Breast ; Estrogens ; Female ; Humans ; Multivariate Analysis ; Prognosis ; Receptors, Estrogen ; Receptors, Progesterone ; Recurrence ; Triple Negative Breast Neoplasms

Plasmacytoma in patients with multiple myeloma usually develops in the advanced stage of the disease. We report herein an atypical case of extramedullary relapse of multiple myeloma that presented as mechanical obstruction of the small bowel in a patient who had achieved complete remission after chemotherapy. A 75-year-old man was diagnosed with multiple myeloma 25 months previously and treated with a bortezomib-containing chemotherapy regimen. He presented for evaluation of abdominal pain. A circumferential mass resulting in mechanical ileus was observed by abdominal computed tomography. Biopsy after surgical resection confirmed the diagnosis of plasmacytoma. The patient was subsequently treated with thalidomide-containing chemotherapy, but he died of disease progression after 6 months. We suggest careful observation of unusual relapses of multiple myeloma in patients who have achieved complete remission after antimyeloma therapy.
Abdominal Pain ; Aged ; Biopsy ; Diagnosis ; Disease Progression ; Drug Therapy ; Humans ; Ileus ; Intestinal Obstruction ; Multiple Myeloma* ; Plasmacytoma ; Recurrence*

Background/Aims: Hepatitis B core antibody (anti-HBc)-positive donors are used as an extended donor pool, and current guidelines recommend the usage of nucleos(t)ide analogues (NAs) as prophylaxis for preventing de novo hepatitis B virus infection (DNH). We analyzed the long-term outcomes of a large cohort of liver transplantation (LT) patients receiving anti-HBc-positive grafts and evaluated the risk of DNH when hepatitis B immunoglobulin (HBIG) monotherapy was used as prophylaxis. We also compared the cost-effectiveness of HBIG and NAs. Methods: We retrospectively reviewed 457 patients with anti-HBc-positive grafts and 898 patients with anti-HBc-negative grafts who underwent LT between January 2001 and December 2018. We compared recipient characteristics according to the anti-HBc status of the donor, and compared the costs of using NAs for the rest of the patient’s life and using HBIG to maintain hepatitis B surface antibody titers above 200 IU/L. Results: The 1-, 5-, and 10-year patient survival rates were 87.7%, 73.5%, and 67.7%, respectively, in patients with anti-HBc-positive grafts, and 88.5%, 77.4%, and 70.3%, respectively, in patients with anti-HBc-negative grafts (P=0.113). Among 457 recipients with anti-HBc-positive grafts, 117 (25.6%) were non-HBV recipients. The overall incidence of DNH was 0.9%. When using HBIG under insurance coverage, the cumulative cost was lower compared with using NA continuously without insurance coverage in Korea. Conclusions Anti-HBc-positive grafts alone do not affect patient survival or graft survival. HBIG monoprophylaxis has good outcomes for preventing DNH, and the patient’s long-term cost burden is low in Korea because of the national insurance system in this cohort.

Background/Aims: Hepatitis B core antibody (anti-HBc)-positive donors are used as an extended donor pool, and current guidelines recommend the usage of nucleos(t)ide analogues (NAs) as prophylaxis for preventing de novo hepatitis B virus infection (DNH). We analyzed the long-term outcomes of a large cohort of liver transplantation (LT) patients receiving anti-HBc-positive grafts and evaluated the risk of DNH when hepatitis B immunoglobulin (HBIG) monotherapy was used as prophylaxis. We also compared the cost-effectiveness of HBIG and NAs. Methods: We retrospectively reviewed 457 patients with anti-HBc-positive grafts and 898 patients with anti-HBc-negative grafts who underwent LT between January 2001 and December 2018. We compared recipient characteristics according to the anti-HBc status of the donor, and compared the costs of using NAs for the rest of the patient’s life and using HBIG to maintain hepatitis B surface antibody titers above 200 IU/L. Results: The 1-, 5-, and 10-year patient survival rates were 87.7%, 73.5%, and 67.7%, respectively, in patients with anti-HBc-positive grafts, and 88.5%, 77.4%, and 70.3%, respectively, in patients with anti-HBc-negative grafts (P=0.113). Among 457 recipients with anti-HBc-positive grafts, 117 (25.6%) were non-HBV recipients. The overall incidence of DNH was 0.9%. When using HBIG under insurance coverage, the cumulative cost was lower compared with using NA continuously without insurance coverage in Korea. Conclusions Anti-HBc-positive grafts alone do not affect patient survival or graft survival. HBIG monoprophylaxis has good outcomes for preventing DNH, and the patient’s long-term cost burden is low in Korea because of the national insurance system in this cohort.