A Rare Periosteal Diaphyseal Lesion of the Ulna

Periosteal lesions of the ulna diaphysis are rare, include a wide spectrum of tumors, and may cause considerable diagnostic problems. Surgical treatment may vary widely, based on an accurate diagnosis. We present the case of a periosteal, extraskeletal low grade myxoid chondrosarcoma of the ulna diaphysis. The surgical therapy included an en-bloc resection with allograft reconstruction. The patient showed a favorable outcome. Careful preoperative evaluation and planning are imperative to obtain a satisfactory oncological and functional outcome, especially with uncommon tumor presentations at rare locations

Metastatic breast carcinoma mimicking a sebaceous gland neoplasm: a case report

<p>Abstract</p> <p>Introduction</p> <p>Breast cancer is common in women and its metastases involve the skin in approximately one quarter of patients. Accordingly, metastatic breast cancer shown to be cutaneous through histology must be distinguished from a wide variety of other neoplasms as well as the diverse morphologic variants of breast cancer itself.</p> <p>Case presentation</p> <p>We report the case of a 61-year-old Caucasian woman with cutaneous metastases of a bilateral ductal breast carcinoma that in histopathological examination mimicked an adnexal neoplasm with sebaceous differentiation.</p> <p>Conclusion</p> <p>Against the background of metastatic breast carcinoma, dermatopathological considerations of sebaceous differentiation of skin lesions are presented and discussed focusing on the rare differential diagnosis of sebaceous carcinoma of the breast.</p

Reproduction in Heteroteuthis dispar (Rüppell, 1844) (Mollusca: Cephalopoda): a sepiolid reproductive adaptation to an oceanic lifestyle

Small cephalopods of the genus Heteroteuthis are the most pelagic members in the family Sepiolidae. This study examines the reproductive biology of Heteroteuthis dispar (Rüppell, 1844), the first such study on any member of the genus, based on 46 specimens (27 females and 19 males) collected during the Mar-Eco cruise in the North Atlantic in the region of the Mid-Atlantic Ridge in 2004, and compares it with reproductive features in the less pelagic members of the family. The unusually large spermatophores of the males have a very small ejaculatory apparatus and cement body, relative to the size of the sperm mass. Females first mate when they are still maturing: a large sperm mass (up to 3.4% of the female body mass), consisting of one to several spermatangia, was found in an internal seminal receptacle of the majority of the females examined regardless of their maturity state. The seminal receptacle has a unique form and position in this species. The receptacle is a thin-walled sac at the posterior end of the visceral mass that is an outpocketing of, and opens into, the visceropericardial coelom. Spermatangia and sperm from the spermatangia apparently enter into the visceropericardial coelom (which is mostly occupied by the ovary) from the seminal receptacle indicating that ova are fertilised internally, a strategy unknown for decapodiform cephalopods (squid and cuttlefish), but present in most octopods. Fecundity of Heteroteuthis dispar (1,100–1,300 oocytes) is much higher than in other sepiolids whereas the egg size (mean max. length ∼1.6 mm) is the smallest within the family. Spawning is continuous (sensu Rocha et al. in Biol Rev 76:291–304, 2001). These and other reproductive traits are discussed as being adaptations to an oceanic lifestyle

Dedifferentiated liposarcoma with leukocytosis. A case report of G-CSF-producing soft-tissue tumors, possible association with undifferentiated liposarcoma lineage

<p>Abstract</p> <p>Background</p> <p>Granulocyte-colony-stimulating factor (G-CSF) functions as a hematopoietic growth factor and it is responsible for leukocytosis. G-CSF-producing tumors associated with leukocytosis include various types of malignancies.</p> <p>Case presentation</p> <p>We report the case of a 72-year-old man with dedifferentiated liposarcoma characterized by dedifferentiated components of malignant fibrous histiocytoma (MFH)-like features in addition to well-differentiated lipoma-like liposarcoma, arising from his upper arm. Preoperative laboratory data showed leukocytosis (103,700/μl). The serum level of G-CSF was also elevated (620 pg/ml [normal, <8 pg/ml]). Nine days after the surgery, the leukocytosis was relieved (WBC; 6,920/μl) and the elevated serum G-CSF level was significantly decreased (G-CSF; 12 pg/ml). One month after the surgery, leukocytosis gradually began to appear again. Three months after the surgery metastatic lung lesions were confirmed, and the patient subsequently died of respiratory problems. In the English literature regarding soft-tissue tumors with leukocytosis, including the current case, we could review a total of 6 cases of liposarcoma with leukocytosis. The subtype of these 6 liposarcoma cases was undifferentiated liposarcoma, comprising dedifferentiated liposarcoma in 4 cases and pleomorphic liposarcoma in 2 cases.</p> <p>Conclusion</p> <p>Since the only other soft-tissue tumor that was associated with leukocytosis was MFH, and since MFH is characterized by the absence of any specific differentiation, we would like to propose a possible association between G-CSF-producing soft-tissue tumors and an undifferentiated liposarcoma lineage, such as dedifferentiated liposarcoma or pleomorphic liposarcoma.</p

Dedifferentiated chondrosarcoma with leukocytosis and elevation of serum G-CSF. A case report

BACKGROUND: G-CSF is known to function as a hematopoietic growth factor and it is known to be responsible for leukocytosis. G-CSF-producing tumors associated with leukocytosis include various types of malignancies. CASE PRESENTATION: We report the case of a 72-year-old man with dedifferentiated chondrosarcoma characterized by dedifferentiated components of malignant fibrous histiocytoma- or osteosarcoma-like features in addition to conventional chondrosarcoma, arising from his pelvic bone. After hemipelvectomy, when local recurrence and metastasis were identified, leukocytosis appeared and an elevated level of serum granulocyte-colony-stimulating factor (G-CSF) was also recognized. The patient died of multiple organ failure 2 months after surgery. Autopsy specimens showed that the histological specimens of the recurrence and metastasis were dedifferentiated components, without any conventional chondrosarcoma components. G-CSF was expressed only in the dedifferentiated components, not in the chondrosarcoma components, immunohistochemically. CONCLUSION: This is the first report of chondrosarcoma, or any other primary bone tumor, with leukocytosis, probably stimulated by tumor-produced G-CSF from the dedifferentiated components

Involvement of RhoA-mediated Ca(2+ )sensitization in antigen-induced bronchial smooth muscle hyperresponsiveness in mice

BACKGROUND: It has recently been suggested that RhoA plays an important role in the enhancement of the Ca(2+ )sensitization of smooth muscle contraction. In the present study, a participation of RhoA-mediated Ca(2+ )sensitization in the augmented bronchial smooth muscle (BSM) contraction in a murine model of allergic asthma was examined. METHODS: Ovalbumin (OA)-sensitized BALB/c mice were repeatedly challenged with aerosolized OA and sacrificed 24 hours after the last antigen challenge. The contractility and RhoA protein expression of BSMs were measured by organ-bath technique and immunoblotting, respectively. RESULTS: Repeated OA challenge to sensitized mice caused a BSM hyperresponsiveness to acetylcholine (ACh), but not to high K(+)-depolarization. In α-toxin-permeabilized BSMs, ACh induced a Ca(2+ )sensitization of contraction, which is sensitive to Clostridium botulinum C3 exoenzyme, indicating that RhoA is implicated in this Ca(2+ )sensitization. Interestingly, the ACh-induced, RhoA-mediated Ca(2+ )sensitization was significantly augmented in permeabilized BSMs of OA-challenged mice. Moreover, protein expression of RhoA was significantly increased in the hyperresponsive BSMs. CONCLUSION: These findings suggest that the augmentation of Ca(2+ )sensitizing effect, probably via an up-regulation of RhoA protein, might be involved in the enhanced BSM contraction in antigen-induced airway hyperresponsiveness

NR4A3 rearrangement reliably distinguishes between the clinicopathologically overlapping entities myoepithelial carcinoma of soft tissue and cellular extraskeletal myxoid chondrosarcoma

Myoepithelial carcinoma of soft tissue (MEC) and cellular extraskeletal myxoid chondrosarcoma (cEMC) share striking similarities. In this paper, we compare ten MECs with five cEMCs. MEC patients had an equal gender distribution. The age range was 15–76 years (mean, 42 years). Tumours were located on extremities, pelvic girdle, vulva and neck. Follow-up, available for nine patients, ranged from 4 to 85 months (mean, 35 months). Five patients were alive without evidence of disease, two were alive with disease and two died 8 months after the initial diagnosis. cEMCs were from three males and two females with an age range of 37–82 years (mean, 57 years); they presented in extremities, shoulder and paravertebral/cervical. Follow-up, available for four patients, ranged from 6 to 220 months (mean, 61 months). All patients were alive, two with recurrences and/or metastases and two without evidence of disease. Morphologically, the distinction between these two entities was difficult since all cases exhibited features typically seen in myoepithelial tumours. Immunohistochemically, MECs expressed pan-keratin (80 %), epithelial membrane antigen (EMA; 57 %), S100 (50 %), alpha-smooth muscle actin (ASMA; 75 %), calponin (67 %) and p63 (25 %). S100 and EMA were expressed in 40 % of cEMC cases respectively with additional immunoreactivity for p63, ASMA and glial fibrillary acidic protein in one case. Pan-keratin was negative in all neoplasms. NR4A3 rearrangement was present in four of four cEMCs and in none of the MECs. In contrast, three of nine (33 %) MECs and four of five (80 %) cEMCs showed an EWSR1 rearrangement. In summary, MECs and cEMCs share clinical, morphological, immunohistochemical and genetic characteristics. The pathognomic rearrangement of NR4A3 is a useful diagnostic feature in identifying cEMCs

Age-related change in brain metabolite abnormalities in autism: a meta-analysis of proton magnetic resonance spectroscopy studies

Abnormal trajectory of brain development has been suggested by previous structural magnetic resonance imaging and head circumference findings in autism spectrum disorders (ASDs); however, the neurochemical backgrounds remain unclear. To elucidate neurochemical processes underlying aberrant brain growth in ASD, we conducted a comprehensive literature search and a meta-analysis of 1H-magnetic resonance spectroscopy (1H-MRS) studies in ASD. From the 22 articles identified as satisfying the criteria, means and s.d. of measure of N-acetylaspartate (NAA), creatine, choline-containing compounds, myo-Inositol and glutamate+glutamine in frontal, temporal, parietal, amygdala-hippocampus complex, thalamus and cerebellum were extracted. Random effect model analyses showed significantly lower NAA levels in all the examined brain regions but cerebellum in ASD children compared with typically developed children (n=1295 at the maximum in frontal, P<0.05 Bonferroni-corrected), although there was no significant difference in metabolite levels in adulthood. Meta-regression analysis further revealed that the effect size of lower frontal NAA levels linearly declined with older mean age in ASD (n=844, P<0.05 Bonferroni-corrected). The significance of all frontal NAA findings was preserved after considering between-study heterogeneities (P<0.05 Bonferroni-corrected). This first meta-analysis of 1H-MRS studies in ASD demonstrated robust developmental changes in the degree of abnormality in NAA levels, especially in frontal lobes of ASD. Previously reported larger-than-normal brain size in ASD children and the coincident lower-than-normal NAA levels suggest that early transient brain expansion in ASD is mainly caused by an increase in non-neuron tissues, such as glial cell proliferation