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Biomedical subjects

Junji Takeyama

Publications and source records attributed to Junji Takeyama.

14 recordsLinked to original sources

PTHrP-independent hypercalcemia with increased proinflammatory cytokines and bone resorption in two children with CD19-negative precursor B acute lymphoblastic leukemia.

Hypercalcemia in childhood acute lymphoblastic leukemia (ALL) is rare and occasionally associated with parathyroid hormone-related protein (PTHrP). However, the pathogenesis of PTHrP-independent hypercalcemia remains unclear. We report two children with precursor B ALL who had marked hypercalcemia (15.8 and 16.6 mg/dl, respectively) and disseminated osteolysis. Serum tumor necrosis factor-alpha (TNF-alpha) and IL-6 were markedly elevated, whereas 1,25(OH)(2) vitamin D(3), intact PTH and PTHrP were decreased or undetected. Analysis of urinary deoxypyridinoline (DPY) or bone biopsy of the osteolytic lesion showed an increased bone resorption, and administration of bisphosphonate improved the hypercalcemia. Patients had ALL with immunophenotype positive for CD10, CD34, and HLA-DR but negative for CD19 and obtained remission with chemotherapy. These findings suggest that increased osteoclastic bone resorption via stimulation with TNF-alpha and IL-6 may be mechanism causing PTHrP-independent hypercalcemia in some patients with precursor B ALL lacking CD19 expression.

Amino Acids↗

Subcutaneous granuloma annulare in a child's palm: a case report.

We present a rare case of subcutaneous lesions arising in the palm of a 4-year-old girl. Histologic examination of the biopsy specimens showed granulomatous inflammation with focal necrobiosis, which was consistent with subcutaneous granuloma annulare. Subcutaneous granuloma annulare is a self-limited disease and treatment is not required, although recurrence and/or multiple lesions are frequent.

Biopsy↗

Delayed separation of the umbilical cord in two siblings with Interleukin-1 receptor-associated kinase 4 deficiency: rapid screening by flow cytometer.

We describe 2 siblings who had interleukin-1 receptor-associated kinase 4 deficiency with a novel mutation in exon 2. They had delayed separation of the umbilical cord. The flow cytometric analysis of monocytic intracellular tumor necrosis factor-alpha production in response to lipopolysaccharide may be a useful method to screen for the disease.

Biomarkers↗

Epstein-Barr virus associated Hodgkin lymphoma in a 9-year-old girl receiving long-term methotrexate therapy for juvenile idiopathic arthritis.

We describe a case of Hodgkin lymphoma developing in a 9-year-old girl with polyarticular, rheumatoid factor-positive juvenile idiopathic arthritis treated with methotrexate (MTX), prednisone, and naproxen for 5 years. Pathologic and molecular analyses revealed that the Hodgkin cells contained Epstein-Barr virus and the viral DNA was monoclonal. She achieved complete remission after MTX withdrawal, chemotherapy, and radiation. To the best of my knowledge, this is the sixth report of Hodgkin lymphoma in patients with juvenile idiopathic arthritis receiving low dose MTX therapy.

Arthritis, Juvenile↗

Spinal hamartoma associated with spinal dysraphism.

OBJECTS: The aims of the study were (1) to review the pathological findings of spinal lipomatous masses associated with congenital spinal dysraphism and (2) to discuss the pathological diagnosis. METHODS: The pathological records of 47 patients at our institution were reviewed, and three illustrative cases were presented. CONCLUSION: Spinal tumorous lesions associated with spinal dysraphism have been traditionally described as lipoma since they are composed mostly of fatty tissue. However, they are different from lipomas arising in other part of the body in that they often contain various tissues of ecto- and mesodermal origin. In our study, we detected such heterotopic components in 24 out of 47 cases. Although they are also similar to teratoma, it is generally accepted that they are malformative lesions which lack neoplastic potential. We therefore should diagnose them as hamartoma rather than lipoma or teratoma.

Child↗

Primary tracheal schwannoma (neurilemoma) in a 9-year-old girl.

The authors report a case of primary tracheal schwannoma (neurilemoma) causing symptoms of airway obstruction in a 9-year-old girl. Computerized tomography scan showed a polypoid intratracheal mass obstructing 73% of the lumen. Partial tracheal resection with primary anastomosis was performed. Histologic analysis showed a benign neurogenic tumor of Schwann cell origin.

Airway Obstruction↗

Rhabdomyomatous mesenchymal hamartoma associated with nasofrontal meningocele and dermoid cyst.

A 1-year-6-month-old girl presented with a subcutaneous tumor of the forehead, which had developed since birth. The preoperative examinations showed nasofrontal bone defect with meningocele and subcutaneous tumor with cyst. The patient underwent excision of the tumor and reconstruction of the bone defect. Histologically, the skin tumor exhibited disordered arrangement of striated muscle fibers among normal dermal components, and the cyst was lined by cornified epithelium with a few hair adnexa and contained lamellated keratin. These findings were consistent with rhabdomyomatous mesenchymal hamartoma (RMH) and dermoid cyst. This is an interesting case of RMH co-existing with nasofrontal meningocele and dermoid cyst in the same area. We suggest embryologic errors as a possible etiology, which is incomplete dysjunction of the neural ectoderm from the cutaneous ectoderm. Failure of insertion of mesoderm between the ectoderms caused the bone defect and the disordered proliferation and differentiation of mesoderm-derived tissue, leading to formation of hamartoma.

Dermoid Cyst↗

Sex steroid hormone receptors in human skin appendage and its neoplasms.

Sex steroids have been postulated to influence pathophysiology of human skin through various skin appendages. The presence of sex steroid receptors has been also reported in adnexal tumors but its details still remained unknown. Therefore, in this study, we immunolocalized sex steroid receptor protein (estrogen receptor (ER)alpha, ERbeta, progesterone receptor (PR)A, PRB and androgen receptor (AR)) in 23 cases of non-pathological skin (male: 10, female: 13) and in 50 cases of skin adnexal tumors (male 24, female 26; 38 benign and 12 malignant). ERalpha immunoreactivity was detected exclusively in basal cells of sebaceous glands of non-pathological skin. AR and PRB immunoreactivity was detected in both differentiated and basal cells of sebaceous gland. AR and ERbeta immunoreactivity was also detected in sebaceous and eccrine sweat glands but not in outer root sheath of hair follicles. In sebaceous gland neoplasms, the number of ERalpha positive cases was significantly lower in skin appendage neoplasms than non-pathological skin. ERbeta immunoreactivity was not detected in any of sebaceous gland neoplasms examined. There were no significant differences in PRA, PRB and AR immunoreactivity between non-pathological sebaceous gland and its neoplasm. In sweat gland neoplasms, the number of AR positive cases was significantly lower in benign neoplasms than their non-pathological counterpart. Therefore sex steroids are considered to play important roles in regulation of non-pathological skin appendage function and pathogenesis and/or development of its neoplasm. In addition, the status of the great majority of sex steroid hormone receptors was maintained throughout the process of neoplastic transformation of skin appendages, except for AR and ERalpha in sweat and sebaceous gland neoplasms.

Adolescent↗

Acute renal failure due to leukemic cell infiltration followed by relapse at multiple extramedullary sites in a child with acute lymphoblastic leukemia.

Acute renal failure due to leukemic infiltration into the kidney is rare in childhood acute lymphoblastic leukemia (ALL). We report here a five year-old boy with ALL who presented acute renal failure caused by leukemic infiltration at onset. Treatment with predonisolone and hemodialysis was effective. However, he showed persistent or repeated relapses at extramedullary sites, such as central nervous system, testis, and pancreas, suggesting that leukemic cells of this patient may have had a high affinity to extramedullary organs. On the basis of previous reports and the experience of this patient, intensive treatment may be needed in ALL children with renal involvement.

Acute Kidney Injury↗

Invasive micropapillary carcinoma of the breast: clinicopathological and immunohistochemical study.

Invasive micropapillary carcinoma (IMPCa) of the breast refers to a unique variant of invasive ductal carcinoma, but its biological behavior has not been elucidated well. We analyzed 16 IMPCa cases (10 pure type, six mixed type). The incidence of IMPCa was 1.0% of all primary breast carcinoma. High nuclear grade (75.0%), as well as poorly differentiated histological grade (81.3%), was frequently seen. Lymph node metastases were evident in 92.9% of the examined cases, and about half of them showed more than 10 positive nodes. Comparison between serially experienced invasive ductal carcinoma, not otherwise specified (IDC-NOS), revealed that both high nuclear grade and poor histological grade were significantly more frequent (P < 0001), there was a lower frequency of positive estrogen receptor/progesterone receptor (P < 0.05, P < 0.01), a higher frequency of HER-2 overexpression (P < 0.025), and more frequent lymph node metastases (P < 0.05) in IMPCa. The comparison between lymph node positive IDC-NOS did not show any statistically significant differences in frequency for positive p53, matrix metalloproteinase protein-2 (MMP-2), vascular endothelial growth factor (VEGF) or E-cadherin. However, IMPCa showed a significantly increased number of blood vessels counted by CD34 immunostains (P < 0.05). These results suggest that IMPCa is, at least, the same or more aggressive than lymph node positive cases of IDC-NOS. Hence, not only the high incidence of lymph node metastases but also distant, blood-borne metastases may be important.

Biomarkers, Tumor↗

Orexin-A expression in human peripheral tissues.

Orexin-A is a neuropeptide present in the brain and is known to regulate feeding and sleeping. In this study, we examined the systemic distribution of orexin-A in human tissues. Immunoreactivity for orexin-A was detected in ganglion cells of the thoracic sympathetic trunk, myenteric plexuses and endocrine cells of the gastrointestinal tract, islet cells of the pancreas and syncytiotrophoblasts and decidual cells of the placenta. In the gastrointestinal tract, orexin-A immunoreactivity was detected in the myenteric plexuses from 26 gestational weeks to birth. In double immunostaining in the pancreas, a great majority of insulin-positive cells was simultaneously positive for orexin-A. mRNA expression for prepro-orexin was also detected in the kidney, adrenal gland, pancreas, placenta, stomach, ileum, colon and colorectal epithelial cells. These results suggest the production of orexin-A in various human peripheral tissues and orexin-A may also play important roles in some peripheral organs.

Adolescent↗

Endocervical-like mucinous borderline tumors of the ovary: clinicopathological features and electron microscopic findings.

Endocervical-like mucinous borderline tumor (EMBT) is a distinct entity of the ovary that seems to be underrecognized. It occurs with relatively high frequency in Japanese women. Compared with intestinal-type mucinous borderline tumor (IMBT), more frequent bilateral occurrence, paucilocular cysts, association with endometriosis, absence of pseudomyxoma but possible association of peritoneal implants and lymph node metastases, and lower mortality rate are the characteristic features. Histologically, it consists of a mixture of two types of epithelium, tall columnar mucinous cells and stratified eosinophilic cells. Electron microscopy revealed that they were endocervical glandlike mucinous cells and ciliated columnar epithelium reminiscent of the fallopian tube. As the mixture of EMBT and serous borderline tumor (seromucinous borderline tumor) occurs, these findings may show that the tumor shows MUllerian origin with two-way differentiation, or differentiation toward endocervical glands with metaplastic features as seen in reactive endocervical lesions.

Adenocarcinoma, Mucinous↗