Primary localized amyloid tumor of the breast with osseous metaplasia

A case of primary localized amyloid tumor of the breast is described. It Is an extremely rare condition and has not been seen in literature in Japan. A 76-year-old woman visited a hospital because of a painless, hard mass of the right breast. A relatively well demarcated, calcified mass was excised under clinical diagnosis of fibroadenoma. Histologically, massive eoslnophilic amorphous material was deposited in breast stroma. It was stained red-orange by Congo red and displayed apple-green birefringence under polarized light. The staining persisted after incubation with KMnO₄ and immunolabeling by immunoglobulin x-light chain antlserum, consistent with the amyloid of AL (Ax) type. Osseous metaplasia with bone marrow cavity, foreign body type giant cells in response to amyloid, and scattered plasma cell infiltration were also recognized. Osseous metaplasia in the breast amyloid tumor has been reported in only one case before. To date, the patient has not developed any clinical or laboratory evidence of systemic amyloidosis or multiple myeloma.     

Gorlin syndrome with ulcerative colitis in a Japanese girl

We present the case of a 14-year-old Japanese girl who had both Gorlin syndrome and ulcerative colitis. She had complained of blood stools for 6 months and severe scoliosis from her infancy. Physical examination revealed multiple nevi, palmar and plantar pits, jaw cysts, and calcification of the falx cerebri, leading to the diagnosis of Gorlin syndrome. Total colonoscopy revealed an edematous and spotty bleeding mucosa extending from the anus to the transverse colon. Histological examination was also compatible with ulcerative colitis. Thus, we diagnosed her as having Gorlin syndrome with ulcerative colitis. Gene analysis revealed a mutation, 1247InsT, in the human patched gene (PTCH), resulting in the truncation of PTCH protein. Since Gorlin syndrome and ulcerative colitis are rare disorders in childhood, this association is interesting, suggesting a correlation between the hedgehog signaling and intestinal disorders.     

Expression of insulin-like growth factor 1 receptor in primary breast cancer: immunohistochemical analysis

Insulin-like growth factor-1 receptor (IGF-1R) has been implicated in regulation in tumor growth. The results of previous studies performed by radioimmunoassay are conflicting, and the prognostic significance of IGF-1R expression in primary breast cancer is still controversial. IGF-1R expression was evaluated in formalin-fixed, paraffin-embedded tissue of 210 primary breast cancer patients by using anti-IGF-1R antibody. The clinicopathologic variables and 5-year disease-free survival were studied, and their correlations between IGF-1R expressions were investigated. IGF-1R overexpression was observed in 43.8% of tumors. IGF-1R overexpression had no correlation with prognosis or with other clinicopathologic parameters, such as age, tumor size, nodal status, histologic grade, hormone receptor status, and human epidermal growth factor 2 status. Though its prognostic value in breast cancer is limited, immunohistochemical evaluation of IGF-1R by using this monoclonal antibody may be useful in translational research using archived material.     

Immunohistochemical study of tyrosine-hydroxylase-positive cells and fibers in the chicken spinal cord.

Tyrosine hydroxylase (TH)-positive cells and fibers were examined by immunohistochemistry in the chick spinal cord. TH-positive cells, which were located in laminae I, V and X, were most frequently found in the rostral part of the cervical spinal cord, with fewer cells being found in more caudal levels of the spinal cord. TH-positive cells located in lamina X, which were bipolar in shape, were mainly found in regions lateral as well as just ventral to the central canal. They had processes reaching to the central canal. The terminals of these cerebrospinal-fluid-contacting cells were oval in shape, and were most frequently found at the ventral wall of the central canal. There were dense clusters of TH-positive fibers in lamina X. A meshwork-like structure of TH-positive fibers was found over the lateral wall of the central canal. A high density of TH-positive fibers was also found in the medial part of laminae V-VII. In lamina IX, small numbers of TH-positive fibers were observed in the lateral motor column of the brachial spinal cord, and in the medial and lateral motor columns of the lumbosacral spinal cord. However, within the medial motor column of the brachial spinal cord TH-positive fibers were densely distributed around somal as well as dendritic profiles. Similar to our previous observations on serotoninergic fibers. TH-positive fibers were also differentially distributed in the ventral horn of the chicken spinal cord: a high density of TH-positive fibers was localized to specific regions of the spinal motor nucleus.     

Association of natural tooth loss with genetic variation at the human matrix Gla protein locus in elderly women

Natural tooth loss represents a major medical issue within the elderly population, since it impairs masticatory function critical for oral intake of essential nutrition. Contribution of genetic factors has been implicated in the determination of natural tooth loss; degree of reduction in number of natural teeth remaining intact (NTI) varies among individuals; thus, heterogeneity in NTI might reflect genetic variation within the population. One candidate gene, the matrix Gla protein gene (MGP), has been implicated in the pathogenesis of bone loss through a repression of bone/tooth formation. We have investigated a possible association between the CA repeat polymorphism at the human MGP gene locus and the NTI in 458 elderly Japanese women. In 916 chromosomes tested, ten alleles of the polymorphic nucleotide repeat were observed (designated A1–A10), among which five alleles were regarded as major alleles to be tested for the association. Twenty-seven women who possessed an A6 allele (164 bp) had significantly higher NTI than the remaining participants (n=431), who did not carry an allele of that size (mean: 10.0 teeth vs 5.6 teeth; P=0.007, Mann-Whitney test). An eight-year longitudinal follow-up study of NTI suggested that the genetic variations at the MGP locus did not affect the rate of tooth loss in the elderly period. These results suggest that genetic variation at the MGP gene locus is associated with some determinants for tooth loss in elderly women.     

Primary rhabdomyosarcoma of the iliac bone in an adult: A case mimicking fibrosarcoma

Primary rhabdomyosarcoma of bone is exceedingly rare. We present a case of rhabdomyosarcoma of the iliac bone in a 32-year-old male. Histologically, the tumour consisted mainly of a uniform proliferation of elongated spindle cells arranged in a herring bone pattern, simulating fibrosarcoma. Focally there was a conventional embryonal pattern with scattered rhabdomyoblasts possessing an eosinophilic cytoplasm. Immunohistochemical studies disclosed expression of muscle markers such as desmin and muscle-specific actin, in both the embryonal and spindle-cell areas and myoglobin only in the embryonal areas. Such histological features are unusual for classical embryonal rhabdomyosarcoma. The anatomical site and age of the patient are also atypical.     

Establishment and characterization of αs1-casein–specific T-cell lines from patients allergic to cow’s milk: Unexpected higher frequency of CD8 T-cell lines

To study cow’s milk allergy at the cellular level, we assessed the reactivity of peripheral blood mononuclear cells from patients allergic to cow’s milk to α_s1-casein, which is one of the major allergens in cow’s milk. Proliferation of the cells to α_s1-casein activation showed a rather weak response. Therefore to understand T-cell reactivity to α_s1-casein in more detail, we prepared α_s1-casein–specific T-cell lines from patients allergic to cow’s milk and established 26 T-cell lines. These T-cell lines could be classified into three groups by analyzing their surface marker expression: those containing predominantly CD4⁺CD8^- T cells, those containing both CD4⁺CD8^- and CD4^-CD8⁺ T cells, and those containing predominantly CD4^-CD8⁺ T cells. The CD8⁺ T cells were obtained at an unexpectedly higher frequency from the patients. These T-cell lines produced interferon-γ and IL-4. These results suggest that CD8⁺ T cells specific for α_s1-casein and CD4⁺ T cells were primed by the stimulation with α_s1-casein in patients allergic to milk and that both T cells may play a key role in the onset, progression of, or recovery from cow’s milk allergy. (J ALLERGY CLIN IMMUNOL 1996;97:1342-9.)     

The value of external morphology in the identification of larval anisakid nematodes: a scanning electron microscope study

We studied larval nematodes of four genera of the Anisakidae using a scanning electron microscope (SEM). The anterior and posterior extremities and cuticular structures of the 3rd-stage larvae (L3) ofAnisakis type I,Pseudoterranova decipiens, Contracaecum type B andHysterothylacium were examined. The 4th-stage larvae (L4) ofAnisakis type I,P. decipiens, recovered after infection into laboratory rats, and the L3 and L4 ofAnisakis type I larvae from human were also examined in the same way. There were generic differences in the shape and size of the lip bulges, external papillary structures, the appearance of the boring tooth, the width and depth of the grooves and ridges of the cuticle and the caudal structures of the L3. InAnisakis type I andP. decipiens L3, changes were seen in the anterior extremity, cuticle and posterior extremity after molting to the L4. Similar changes can be expected in larvae infecting man. The L4 ofAnisakis type I from rat and man were similar, while the L4 ofAnisakis type I andP. decipiens showed differences. These ultrastructural differences might be of value in the identification of fragments recovered during endoscopy in man.