Rhabdomyosarcoma with epithelioid morphology: A challenging cytologic diagnosis in a pleural effusion

Rhabdomyosarcomas (RMS) are rare malignant skeletal muscle tumors that present more commonly in pediatric populations. The WHO currently classifies RMS into four types, embryonal, alveolar, pleomorphic, and spindle cell/sclerosing variants. Epithelioid rhabdomyosarcoma (EpiRMS) is another rare, recently described subtype of RMS presenting in older patients with a male predominance and has a rapidly progressive clinical course with frequent metastases. EpiRMS closely mimics poorly differentiated carcinoma or melanoma, demonstrating discohesive large epithelioid cells with abundant eosinophilic cytoplasm, frequent glassy cytoplasmic inclusions, large vesicular nuclei, and prominent nucleoli. We present a case of metastatic rhabdomyosarcoma with features reminiscent of EpiRMS presenting as a pleural effusion, closely followed by an inguinal lymph node biopsy. The malignant cells in the pleural fluid were diffusely positive for desmin, negative for MyoD1, myogenin, S100 and SOX10, and retained INI-1 expression. Subsequent lymph node biopsy demonstrated identical malignant epithelioid cells that were positive for desmin, myoD1 and myogenin, and a cytological diagnosis of metastatic rhabdomyosarcoma, favor epithelioid rhabdomyosarcoma was given considering the concurrent lymph node biopsy morphology and immunoprofile. A diagnosis of rhabdomyosarcoma, though rare and challenging, should not be overlooked when considering malignant cells with an epithelioid morphology in cytology specimens

Functional reconstruction of the glenoid fossa utilizing a pedicled temporal osteomuscular flap

Current techniques in management of end stage pathology of the temporomandibular joint (TMJ) include the use of alloplastic joint reconstruction. A polyethylene glenoid fossa prosthesis is a necessity of this treatment as it provides a stable platform for function of the metal alloy condylar head. Additionally, the fossa prosthesis limits superior and posterior movement of the reconstructed joint which prevents complications such as migration of the condylar prosthesis into the middle cranial fossa and ear, ankylosis, and pain. When a pathologic process affects the glenoid fossa alone, alloplastic joint reconstruction becomes a less desirable treatment option. Lack of osseous structure along the temporal bone and zygomatic arch can impact the surgeon\u27s ability to fixate a glenoid fossa prosthesis. Additionally, resection of an uninvolved condylar head in situations where there is no advanced pathology would provide a functional solution, but may be overly aggressive and potentially unnecessary. The following is our experience with utilizing a pedicled temporal osteomuscular flap to reconstruct an acquired defect of the glenoid fossa in a 42-year-old male with a diffuse-type tenosynovial giant cell tumor. In this case the mandibular condyle was not affected by the pathology

Immunohistochemical characteristics of Renomedullary interstitial cell tumor: a study of 41 tumors with emphasis on differential diagnosis of mesenchymal neoplasms

Renomedullary interstitial cell tumors (RMICT) are almost always incidentally identified either at autopsy or resection of the kidney for other reasons. However, rare cases have been reported which are large, resulting in a clinical mass. The immunohistochemical phenotype of usual, incidental RMICT using modern soft tissue tumor markers in is largely unknown, however, providing little information to aid in classification of larger or atypical tumors. We retrieved 41 RMICTs from 36 patients, and studied pathologic characteristics including morphology, immunohistochemistry (S100, keratin AE1/AE3, smooth muscle actin, desmin, estrogen and progesterone receptors, calponin, CD34, CD35), and histochemical staining. Data collected included age, gender, tumor size, laterality, and indication for kidney examination. RMICTs (n = 41) were identified in 23 men and 13 women, with mean age 57 years (range 24–83), tumor sizes ranged from <1 to 13 mm (median 4 mm). Kidneys were resected for 32 tumors, 1 chronic pyelonephritis, 1 trauma, and 2 autopsies. All (41, 100%) had entrapped renal tubules, 5 (12%) of which included cystic or dilated tubules. Most (35, 85%) had collagenous fibers, all of which were negative for Congo red. RMICT demonstrates a largely negative immunohistochemical phenotype with weak to moderate labeling for smooth muscle actin and calponin that is substantially less than myofibroblastic lesions. Positive staining for estrogen and progesterone receptor is common (61%), which could overlap with mixed epithelial and stromal tumor and other entities; however, staining is typically weak. CD34 is usually negative, with occasional weak labeling, in contrast to solitary fibrous tumor

Inflammatory Leiomyosarcoma and Histiocyte-rich Rhabdomyoblastic Tumor : a clinicopathological, immunohistochemical and genetic study of 13 cases, with a proposal for reclassification as Inflammatory Rhabdomyoblastic Tumor

Inflammatory leiomyosarcoma (ILMS), defined as a malignant neoplasm showing smooth muscle differentiation, a prominent inflammatory infiltrate, and near-haploidization , is a very rare soft tissue tumor with a generally favorable prognosis. The morphologic features of histiocyte-rich rhabdomyoblastic tumor (HRRMT) are similar to those of ILMS, although this lesion shows by definition a skeletal muscle phenotype. Recent gene expression profiling and immunohistochemical studies have also suggested that ILMS and HRRMT may be related. We studied the clinicopathologic, immunohistochemical and genetic features of four cases previously classified as ILMS and nine classified as HRRMT. Tumors from both groups tended to occur in the deep soft tissues of the extremities of young to middle-aged males and exhibited indolent behavior. Morphologically, all were well-circumscribed, often encapsulated, and showed a striking histiocyte-rich inflammatory infiltrate admixed with variably pleomorphic tumor cells showing spindled and epithelioid to rhabdoid morphology, eosinophilic cytoplasm, and prominent nucleoli, but few, if any, mitotic figures. Immunohistochemically, the tumor cells expressed desmin, alpha-smooth muscle actin, and the rhabdomyoblastic markers PAX7, MyoD1, and myogenin. H-caldesmon expression was absent in all cases, using the specific h-CD antibody. Karyotypic study (1 HRRMT) and genome-wide copy number analysis (7 HRRMT, OncoScan SNP assay), revealed near-haploidization in four cases, with subsequent genome doubling in one, an identical phenotype to that seen in ILMS. We propose reclassification of ILMS and HRRMT as inflammatory rhabdomyoblastic tumor , a name which accurately describes the salient morphologic and immunohistochemical features of this distinctive tumor, as well as its intermediate (rarely metastasizing) clinical behavior

Pseudosarcomatous myofibroblastic proliferations of the genitourinary tract are genetically different from nodular fasciitis and lack USP6, ROS1 and ETV6 gene rearrangements

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/145266/1/his13526_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/145266/2/his13526.pd

Multicenter randomized trial of carpal tunnel release with ultrasound guidance versus mini-open technique

BACKGROUND: Comparative studies of carpal tunnel release with ultrasound guidance (CTR-US) vs. mini-open CTR (mOCTR) are limited, prompting development of this randomized trial to compare efficacy and safety of these techniques. RESEARCH DESIGN AND METHODS: Patients were randomized (2:1) to CTR-US or mOCTR, treated by experienced hand surgeons (median previous cases: 12 CTR-US; 1000 mOCTR), and followed for 3 months. RESULTS: Among 149 randomized patients, 122 received CTR-US ( CONCLUSIONS: The efficacy and safety of CTR-US were comparable to mOCTR despite less previous surgical experience with CTR-US. The choice of CTR technique should be determined by shared decision-making between patient and physician. CLINICAL TRIAL REGISTRATION: www.clinicaltrials.gov identifier is NCT05405218

Steady-state modulation of voltage-gated K+ channels in rat arterial smooth muscle by cyclic AMP-dependent protein kinase and protein phosphatase 2B

Voltage-gated potassium channels (Kv) are important regulators of membrane potential in vascular smooth muscle cells, which is integral to controlling intracellular Ca2+ concentration and regulating vascular tone. Previous work indicates that Kv channels can be modulated by receptor-driven alterations of cyclic AMP-dependent protein kinase (PKA) activity. Here, we demonstrate that Kv channel activity is maintained by tonic activity of PKA. Whole-cell recording was used to assess the effect of manipulating PKA signalling on Kv and ATP-dependent K+ channels of rat mesenteric artery smooth muscle cells. Application of PKA inhibitors, KT5720 or H89, caused a significant inhibition of Kv currents. Tonic PKA-mediated activation of Kv appears maximal as application of isoprenaline (a β-adrenoceptor agonist) or dibutyryl-cAMP failed to enhance Kv currents. We also show that this modulation of Kv by PKA can be reversed by protein phosphatase 2B/calcineurin (PP2B). PKA-dependent inhibition of Kv by KT5720 can be abrogated by pre-treatment with the PP2B inhibitor cyclosporin A, or inclusion of a PP2B auto-inhibitory peptide in the pipette solution. Finally, we demonstrate that tonic PKA-mediated modulation of Kv requires intact caveolae. Pre-treatment of the cells with methyl-β-cyclodextrin to deplete cellular cholesterol, or adding caveolin-scaffolding domain peptide to the pipette solution to disrupt caveolae-dependent signalling each attenuated PKA-mediated modulation of the Kv current. These findings highlight a novel, caveolae-dependent, tonic modulatory role of PKA on Kv channels providing new insight into mechanisms and the potential for pharmacological manipulation of vascular tone

Erratum: Sequence data and association statistics from 12,940 type 2 diabetes cases and controls.

This corrects the article DOI: 10.1038/sdata.2017.179

Cross Section and Transverse Single-Spin Asymmetry of η\eta Mesons in p↑+pp^{\uparrow}+p Collisions at s=200\sqrt{s}=200 GeV at Forward Rapidity

We present a measurement of the cross section and transverse single-spin asymmetry ($A_N$) for $\eta$ mesons at large pseudorapidity from $\sqrt{s}=200$~GeV $p^{\uparrow}+p$ collisions. The measured cross section for $0.5<p_T<5.0$~GeV/$c$ and $3.0<|\eta|<3.8$ is well described by a next-to-leading-order perturbative-quantum-chromodynamics calculation. The asymmetries $A_N$ have been measured as a function of Feynman-$x$ ($x_F$) from $0.2<|x_{F}|<0.7$, as well as transverse momentum ($p_T$) from $1.0<p_T<4.5$~GeV/$c$. The asymmetry averaged over positive $x_F$ is $\langle{A_{N}}\rangle=0.061{\pm}0.014$. The results are consistent with prior transverse single-spin measurements of forward $\eta$ and $\pi^{0}$ mesons at various energies in overlapping $x_F$ ranges. Comparison of different particle species can help to determine the origin of the large observed asymmetries in $p^{\uparrow}+p$ collisions.Comment: 484 authors, 13 pages, 11 figures, 4 tables, 2008 data. v2 is version accepted by Phys. Rev. D. Plain text data tables for the points plotted in figures for this and previous PHENIX publications are (or will be)publicly available at http://www.phenix.bnl.gov/papers.htm