Plasmapheresis as therapeutic option in a 16-year-old with EVALI: A case report.

Since 2019 when a cluster of cases with acute respiratory distress syndrome (ARDS) associated with e-cigarettes in the United States was reported, there have been increasing numbers of reports. Electronic-cigarette or Vaping Use-associated Lung Injury (EVALI) represents a recent entity of respiratory clinical syndromes, primarily in young adults. We report a previously healthy 16-year-old boy who developed severe ARDS following a brief nonspecific prodromal phase after excessive consumption of e-cigarettes. Despite maximum intensive care therapy, including several weeks of venovenous extracorporeal membrane oxygenation, plasmapheresis and repeated administration of immunoglobulins seemed the only way to achieve therapeutic success. Although many case reports have been published, to our knowledge, there are none to date on the therapeutic use of plasmaphoresis in severe EVALI. This case highlights the clinical features of EVALI and the diagnostic dilemma that can arise with EVALI occurring against the background of an expired SARS-CoV-2 infection, with a paediatric inflammatory syndrome (PIMS) as differential diagnosis. EVALI is a diagnosis of exclusion, and the medical history of vaping and e-cigarette use can provide valuable clues. Ethical approval for this case report (protocol number 23-145 RS) was provided by the Ethical Committee of the Department of Medicine, Philipps-Universität Marburg, Germany on 13th of June 2023. Written informed consent to publish this case and the associated images was obtained from the patient and his mother.

The value of contrast-enhanced ultrasound in percutaneous biopsy of retroperitoneal masses.

PURPOSE: To evaluate the diagnostic yield of contrast-enhanced ultrasound (CEUS)-guided biopsy of retroperitoneal masses (RMs). MATERIALS AND METHODS: Between 2006 and 2023, 87 patients presented at our US center for biopsy of an RM. In all biopsies, CEUS was performed prior to the intervention. The technical success rate of biopsy, the presence of diagnostic tissue in solid tumor biopsy samples, the accuracy of the biopsy and the occurrence of post-interventional complications were evaluated. RESULTS: A US-guided biopsy could be conducted in 84/87 cases (96.6%). In 3/87 cases (3.4%), US-guided biopsy was impossible because the planned needle path was obstructed by vital structures. Of 84 lesions, 80 (95.2%) were solid lesions, and 4 (4.8%) were lesions containing fluid. In all solid tumors, 80/80 (100%), diagnostic vital tissue was successfully obtained. CEUS-guided biopsy showed a sensitivity of 93.2%, a specificity of 100%, a positive predictive value of 100%, a negative predictive value of 72.2%, and a diagnostic accuracy of 94.2% for the differentiation between malignant and benign RMs. In one of the 84 cases (1.2%), there was a complication of postinterventional abdominal pain. CONCLUSION: Percutaneous CEUS-guided biopsy is a safe procedure with a high diagnostic yield and a low complication rate.

Acoustic Radiation Force Impulse (ARFI) Elastography of Focal Splenic Lesions: Feasibility and Diagnostic Potential.

PURPOSE: Nontraumatic focal splenic lesions (FSL) are rare, and the need for tissue diagnosis must be weighed against the very high risk of bleeding after a splenic biopsy. The aim of this study was to explore the feasibility and diagnostic potential of acoustic radiation force impulse (ARFI) elastography as a noninvasive method for different benign and malignant FSLs. No human studies on the elastographic characteristics of FSL exist. METHODS: This was a retrospective analysis of 34 patients with FSLs, who underwent abdominal B-mode ultrasound (B-US), contrast-enhanced ultrasound (CEUS), and standardized ARFI examinations between October 2021 and December 2022 at our university hospital. The inclusion criteria were: (i) FSL size ≥ 1 cm; (ii) 10 valid ARFI measurements of the FSL, as well as of the normal splenic parenchyma (NSP) as an in vivo reference; and (iii) diagnostic confirmation of FSL etiology based on histological examination (8/34; 23.5%) or clinical evaluation, which included a clinical and sonographic follow-up (FU), CEUS morphology, and/or morphology on cross-sectional imaging (26/34; 76.5%). CEUS was performed on all patients and the FSLs were classified according to the current guidelines; cross-sectional imaging was available for 29/34 (85.3%). The mean FU duration was 25.8 ± 30.5 months. The mean ARFI velocity (MAV) of the FSL (MAVL), the NSP (MAVP), and the ratio of the MAVL to the MAVP (MAVL/P) were calculated and compared. RESULTS: Of the 34 FSLs, 13 (38.2%) were malignant (mFSL) and 21 (61.8%) were benign (bFSL). The MAVL of all 34 FSLs (2.74 ± 0.71 m/s) was lower than the MAVP (3.20 ± 0.59 m/s), p = 0.009, with a mean MAVL/P ratio of 0.90 ± 0.34. No significant differences in the MAVL were observed between the mFSL (2.66 ± 0.67 m/s) and bFSL (2.79 ± 0.75 m/s). There were also no significant differences between the MAVP in patients with mFSL (3.24 ± 0.68 m/s) as compared to that in the patients with bFSL (3.18 ± 0.55 m/s). Likewise, the MAV L/P ratio did not differ between the mFSL (0.90 ± 0.41 m/s) and bFSL (0.90 ± 0.30 m/s) groups. CONCLUSION: ARFI elastography is feasible in evaluating the stiffness of FSLs. The lesions' stiffness was lower than that of the NSP, regardless of the FSL etiology. However, differentiation between benign and malignant FSL with the help of this elastographic method does not appear possible. Larger prospective studies are needed to validate these findings.

High EVI1 and PARP1 expression as favourable prognostic markers in high-grade serous ovarian carcinoma.

BACKGROUND: Mechanisms of development and progression of high-grade serous ovarian cancer (HGSOC) are poorly understood. EVI1 and PARP1, part of TGF-ß pathway, are upregulated in cancers with DNA repair deficiencies with DNA repair deficiencies and may influce disease progression and survival. Therefore we questioned the prognostic significance of protein expression of EVI1 alone and in combination with PARP1 and analyzed them in a cohort of patients with HGSOC. METHODS: For 562 HGSOC patients, we evaluated EVI1 and PARP1 expression by immunohistochemical staining on tissue microarrays with QuPath digital semi-automatic positive cell detection. RESULTS: High EVI1 expressing (> 30% positive tumor cells) HGSOC were associated with improved progression-free survival (PFS) (HR = 0.66, 95% CI: 0.504-0.852, p = 0.002) and overall survival (OS) (HR = 0.45, 95% CI: 0.352-0.563, p < 0.001), including multivariate analysis. Most interestingly, mutual high expression of both proteins identifies a group with particularly good prognosis. Our findings were proven technically and clinically using bioinformatical data sets for single-cell sequencing, copy number variation and gene as well as protein expression. CONCLUSIONS: EVI1 and PARP1 are robust prognostic biomarkers for favorable prognosis in HGSOC and imply further research with respect to their reciprocity.

Pulmonary Cystic Echinococcosis in Contrast-enhanced Ultrasound - A Case Report.

Aim of the study: Pulmonary cystic echinococcosis is a parasitic infection transmitted by dogs and occurring in livestock-raising areas. It is included among the neglected tropical diseases, according to the World Health Organization. Imaging plays a pivotal role in the diagnosis of this disease. While cross-sectional imaging modalities such as computed tomography and magnetic resonance imaging are preferred, lung ultrasound may be another feasible technique. Case description: We report a case of pulmonary cystic echinococcosis in a 26-year-old woman who was examined by contrast-enhanced ultrasound, which showed marked annular enhancement around the hydatid cyst, mimicking a superinfected cyst. Conclusions: Contrast-enhanced ultrasound examination in pulmonary cystic echinococcosis should be studied in a larger population to determine the value of additional contrast administration. In the present case report, no superinfected echinococcal cyst was seen despite marked annular contrast enhancement.

Inflammatory Pseudotumor of the Omentum in Contrast-enhanced Ultrasound.

Aim of the study: Inflammatory pseudotumor is a rare benign tumor that can occur at various body sites. Due to its rare occurrence and histological variety radiological data is heterogeneous and limited. Case description: We present a case of a 71-year-old man with inflammatory pseudotumor of the omentum. Contrast-enhanced ultrasound perfusion pattern showed homogeneous, isoechoic enhancement in the arterial phase with a washout phenomenon in the parenchymal phase, mimicking a peritoneal carcinomatosis. Conclusions: Inflammatory pseudotumor represents a rare, but important benign differential diagnostic option when considering a malignant disorder. Contrast-enhanced ultrasound is helpful in identifying vital tissue for a targeted biopsy for subsequent histological examination that is essential for the exclusion of malignancy.

Nodal lymphangiogenesis and immunophenotypic variations of sinus endothelium in sentinel and non-sentinel lymph nodes of invasive breast carcinoma.

Several studies have demonstrated the de novo formation of lymphatic vessels or the reorganization of lymphatic sinus in tumor-draining lymph nodes, partly preceding the detection of lymphatic metastases. This "lymphovascular niche"is supposed to facilitate the survival of metastatic tumor cells. Few studies on nodal lymphangiogenesis in invasive breast cancer (BC) have been published, not considering tumor-free sentinel lymph nodes (SLN) and tumor types. Specimens of SLN and/ or non-SLN (NSLN) of 95 patients with BC were examined immunohistochemically for expression of the lymphatic endothelial marker D2-40 (podoplanin) on lymphatic vessels and the subcapsular sinus. The number of D2-40-positive lymph vessels in metastases was evaluated with two morphometric methods (Chalkley count and number per HPF). Data was explored with respect to TNM parameters, grading, tumor type, size of metastasis, lymph vessel number and hormone receptor/HER2 status with appropriate statistical tests. Lymphangiogenesis was detected exclusively in and around BC metastases with both methods for lymph vessel quantification being equivalent. Lymph vessel number correlated with the size of metastases, being significantly higher in larger metastases (p < 0.001). There was no significant statistical difference with respect to tumor types. Intranodal lymphangiogenesis could not be verified by D2-40 staining in any of the tumor-free lymph nodes examined. However, D2-40 was frequently detected in sinus endothelial/virgultar cells of the subcapsular sinus, partly with strong uniform positivity. Staining intensity and stained proportion of the subcapsular sinus were markedly heterogeneous, significantly correlating with each other both in SLN and NSLN (p < 0.001). A higher proportion of D2-40 stained subcapsular sinus in SLN was significantly associated with worse overall survival (p = 0.0036) and an independent prognostic parameter in multivariate analysis (p = 0.033, HR 2.87). Further studies are necessary to elucidate the biological and clinical significance of the observed immunophenotypic variations of nodal sinus endothelium.

[Immunohistology in breast diagnostics : Strategies for efficient diagnostics]. / Immunhistologie in der Mammadiagnostik : Strategien für effiziente Diagnostik.

Immunohistological examinations are useful for the histopathological diagnosis of breast carcinoma in various clinical situations. This review article aims to summarize the different immunohistological options. A distinction is made between diagnostic, prognostic, and predictive markers. Especially when a therapeutic decision results from the immunohistological expression pattern, a quantitative, quality-controlled, and validated diagnostic approach is essential.This is relevant, for example, for the classical markers ER, PR, and HER2, but also for Ki-67 and additional markers such as PD-L1. This article provides a practice-oriented summary of the most important immunohistochemical markers in routine breast cancer diagnosis and for the distinction of malignant findings from benign alterations or precursor lesions.

pT3 colorectal cancer revisited: a multicentric study on the histological depth of invasion in more than 1000 pT3 carcinomas-proposal for a new pT3a/pT3b subclassification.

BACKGROUND: Pathological TNM staging (pTNM) is the strongest prognosticator in colorectal carcinoma (CRC) and the foundation of its post-operative clinical management. Tumours that invade pericolic/perirectal adipose tissue generally fall into the pT3 category without further subdivision. METHODS: The histological depth of invasion into the pericolic/perirectal fat was digitally and conventionally measured in a training cohort of 950 CRCs (Munich). We biostatistically calculated the optimal cut-off to stratify pT3 CRCs into novel pT3a (≤3 mm)/pT3b (>3 mm) subgroups, which were then validated in two independent cohorts (447 CRCs, Bayreuth/542 CRCs, Mainz). RESULTS: Compared to pT3a tumours, pT3b CRCs showed significantly worse disease-specific survival, including in pN0 vs pN+ and colonic vs. rectal cancers (DSS: P < 0.001, respectively, pooled analysis of all cohorts). Furthermore, the pT3a/pT3b subclassification remained an independent predictor of survival in multivariate analyses (e.g. DSS: P < 0.001, hazard ratio: 4.41 for pT3b, pooled analysis of all cohorts). While pT2/pT3a CRCs showed similar survival characteristics, pT3b cancers remained a distinct subgroup with dismal survival. DISCUSSION: The delineation of pT3a/pT3b subcategories of CRC based on the histological depth of adipose tissue invasion adds valuable prognostic information to the current pT3 classification and implementation into current staging practices of CRC should be considered.

Presentation of Chloromas in B-Mode Ultrasound and Contrast-Enhanced Ultrasound.

Chloromas, also referred to as myeloid sarcomas, describe rare extramedullary tumor aggregates of malignant myeloid progenitor cells. The aim of this study was investigate the diagnostic features and characteristics of chloromas using contrast-enhanced ultrasound (CEUS). Between July 2007 and April 2021, 15 patients with 20 myeloid neoplasms and suspected chloroma manifestations were examined using B-mode US (B-US) and CEUS. Clinical data and B-US (echogenicity, border, size) and CEUS (hyper-, iso-, hypo- or complex enhancement) characteristics were retrospectively analyzed. Absolute and relative frequencies were determined. In B-US, the chloromas were most frequently hypo-echoic (n = 15, 75%). In addition, a hyperechoic (n = 2, 10%) or echocomplex (n = 3, 15%) presentation was observed. On CEUS, 7 chloromas (35%) had an arterial hyperenhancement, 8 (40%) an iso-enhancement and 3 (15%) a complex enhancement. Two chloromas (10%) did not exhibit any enhancement. We describe for the first time CEUS and B-US patterns of chloromas. They are typically hypo-echoic on B-US and have a strong iso- or hyperenhancement on CEUS, which may help in the differential diagnosis of some unclear masses (e.g., hematoma, abscess) in patients with myeloid neoplasias. Nevertheless, histology is necessary for a reliable diagnosis.

How VEGF-A and its splice variants affect breast cancer development - clinical implications.

BACKGROUND: Altered expression levels and structural variations in the vascular endothelial growth factor (VEGF) have been found to play important roles in cancer development and to be associated with the overall survival and therapy response of cancer patients. Particularly VEGF-A and its splice variants have been found to affect physiological and pathological angiogenic processes, including tumor angiogenesis, correlating with tumor progression, mostly caused by overexpression. This review focuses on the expression and impact of VEGF-A splice variants under physiologic conditions and in tumors and, in particular, the distribution and role of isoform VEGF165b in breast cancer. CONCLUSIONS AND PERSPECTIVES: Many publications already highlighted the importance of VEGF-A and its splice variants in tumor therapy, especially in breast cancer, which are summarized in this review. Furthermore, we were able to demonstrate that cytoplasmatic VEGFA/165b expression is higher in invasive breast cancer tumor cells than in normal tissues or stroma. These examples show that the detection of VEGF splice variants can be performed also on the protein level in formalin fixed tissues. Although no quantitative conclusions can be drawn, these results may be the starting point for further studies at a quantitative level, which can be a major step towards the design of targeted antibody-based (breast) cancer therapies.

The Value of Contrast-Enhanced Ultrasound (CEUS) in the Detection of Perfusion Disturbances in Abdominal Wall Hernias Compared with Surgical and Histological Assessment.

Purpose: This study aimed to evaluate the value of contrast-enhanced ultrasound (CEUS) in the evaluation of perfusion disturbance in irreducible abdominal wall hernias (AWHs). Methods: From 2006 to 2018, 50 patients with an irreducible AWH were examined using B-mode ultrasound (B-US) and CEUS. The ultrasound findings were correlated with subsequent surgical and histological results. The presence of non-enhanced areas (NEAs) in hernia contents on CEUS and the presence of non-perfused areas (NPAs) on surgical and histological evaluation were analyzed retrospectively. Results: On CEUS, 13/50 hernia contents (26.0%) revealed NEAs during complete CEUS examination and 37/50 (74.0%) revealed no NEAs during CEUS examination. On surgical and histological evaluation, NPAs in hernia contents were identified in 11/13 cases (93.3%) with NEAs on CEUS. CEUS was found to have a sensitivity of 100.0%, a specificity of 94.9%, a positive predictive value of 84.6%, and a negative predictive value of 100.0% for the identification of perfusion disturbance in AWHs. Conclusions: The findings of this study demonstrate that using CEUS as an imaging method may be helpful for evaluating the perfusion of hernia contents in incarcerated AWHs. On CEUS, the presence of NEAs may suggest perfusion disturbance in hernia contents.

Perfusion Patterns of Peripheral Pulmonary Granulomatous Lesions Using Contrast-Enhanced Ultrasound (CEUS) and Their Correlation with Immunohistochemically Detected Vascularization Patterns.

PURPOSE: To describe the perfusion patterns of peripheral pulmonary granulomatous lesions (PPGLs) by contrast-enhanced ultrasound (CEUS) and their correlation with vascularization patterns (VPs) represented by immunohistochemical (CD34) endothelial staining. PATIENTS AND METHODS: From January 2007 until September 2020, 10 consecutive patients with histologically confirmed PPGLs were investigated by CEUS. The time to enhancement, classified as early pulmonary-arterial (PA) pattern of enhancement versus delayed bronchial-arterial (BA) pattern of enhancement, the extent of enhancement, classified as marked or reduced, the homogeneity of enhancement, classified as homogeneous or inhomogeneous, and the decrease of enhancement, classified as rapid washout (<120 seconds) or a late washout (≥120 seconds), were analyzed retrospectively. Furthermore, the tissue samples from the study patients and as a control group, 10 samples of normal lung tissue obtained by autopsy, and 10 samples of lung tissue with acute pneumonia obtained by autopsy were immunohistochemically stained with CD34 antibody. The presence of avascular areas (AAs) and the VPs were evaluated in all tissue samples. RESULTS: On CEUS, all PPGLs showed a reduced inhomogeneous BA pattern of enhancement and a rapid washout (<120 seconds). On CD34 staining, all PPGLs showed central AAs in granulomas and a chaotic VP similar to angiogenesis in lung tumors. The lung tissue in control groups revealed on CD34 staining a regular alveolar VP. CONCLUSION: The PPGLs on CEUS show an identical perfusion pattern similar to those of malignant lesions. Furthermore, for the first time, neoangiogenesis was demonstrated as a histopathological correlate to BA pattern of enhancement on CEUS.

Expression of Estrogen Receptor Alpha and Evaluation of Histological Degeneration Scores in Fibroblasts of Hypertrophied Ligamentum Flavum: A Qualitative Study.

The most common spinal disorder in elderly is lumbar spinal stenosis (LSS), resulting partly from ligamentum flavum (LF) hypertrophy. Its pathophysiology is not completely understood. The present study wants to elucidate the role of estrogen receptor α (ER α) in fibroblasts of hypertrophied LF. LF samples of 38 patients with LSS were obtained during spinal decompression. Twelve LF samples from patients with disk herniation served as controls. Hematoxylin & Eosin (H&E) and Elastica stains and immunohistochemistry for ER α were performed. The proportions of fibrosis, loss and/or degeneration of elastic fibers and proliferation of collagen fibers were assessed according to the scores of Sairyo and Okuda. Group differences in the ER α and Sairyo and Okuda scores between patients and controls, male and female sex and absence and presence of additional orthopedic diagnoses were assessed with the Mann-Whitney U test. There was a tendency towards higher expression of ER α in LF fibroblasts in the hypertrophy group (p = 0.065). The Sairyo and Okuda scores were more severe for the hypertrophy group but, in general, not statistically relevant. There was no statistically relevant correlation between the expression of ER α and sex (p = 0.326). ER α expression was higher in patients with osteochondrosis but not statistically significant (p = 0.113). In patients with scoliosis, ER α expression was significantly lower (p = 0.044). LF hypertrophy may be accompanied by a higher expression of ER α in fibroblasts. No difference in ER α expression was observed regarding sex. Further studies are needed to clarify the biological and clinical significance of these findings.

Perfusion Patterns of Peripheral Organizing Pneumonia (POP) Using Contrast-Enhanced Ultrasound (CEUS) and Their Correlation with Immunohistochemically Detected Vascularization Patterns.

Purpose: To describe the perfusion patterns of peripheral organizing pneumonia (POP) by contrast-enhanced ultrasound (CEUS) and their correlation with vascularization patterns (VPs) represented by immunohistochemical CD34 endothelial staining. Methods: From October 2006 until December 2020, 38 consecutive patients with histologically confirmed POPs were standardized-examined by CEUS. The time to enhancement (TE; classified as an early pulmonary-arterial [PA] pattern of enhancement vs. delayed bronchial-arterial [BA] pattern of enhancement), the extent of enhancement (EE; classified as marked or reduced), the homogeneity of enhancement (HE; classified as homogeneous or inhomogeneous), and the decrease of enhancement (DE; classified as rapid washout [<120s] or late washout [≥120s]) were evaluated retrospectively. Furthermore, tissue samples from the study patients were immunohistochemically stained with CD34 antibody. The presence of avascular areas (AAs) and the VPs were evaluated in all tissue samples. Results: The majority of POPs showed a BA pattern of enhancement (71.1%), an isoechoic marked enhancement (76.3%), and an inhomogeneous enhancement (81.6%). A rapid DE was observed in 50.0% of cases. On CD34 staining, all POPs had a chaotic VP, indicating BA neoangiogenesis. AAs (abscess, necrosis, hemorrhage) were identified in (41.9%) cases with an inhomogeneous enhancement on CEUS. Conclusion: On CEUS, POPs predominantly revealed a marked inhomogeneous BA pattern of enhancement with a rapid washout in 50% of cases. Furthermore, we demonstrated that the presence of a PA pattern of enhancement, found in 28.9% of POPs, did not exclude a BA neoangiogenesis as an important feature of chronic inflammatory and malignant processes.

Prognostic relevance of the loss of stromal CD34 positive fibroblasts in invasive lobular carcinoma of the breast.

CD34+ fibroblasts are constitutive stromal components of virtually all organs, including the mammary stroma, being involved in matrix synthesis, antigen presentation, and tumor-associated stromal remodeling. The most common subtype of invasive breast carcinoma, invasive carcinoma of no special type (IBC-NST), is known for its stromal loss of CD34+ fibroblasts while acquiring alpha smooth muscle actin-positive (α-SMA+) myofibroblasts, i.e., cancer-associated fibroblasts (CAF), whereas invasive lobular carcinoma (ILC) displays partial preservation of CD34+ fibroblasts. The aim of this study was to evaluate the prognostic relevance of stromal CD34+ fibroblasts and α-SMA+ myofibroblasts in an extended collection of ILC. A total of 133 cases of ILC, primarily resected between 1996 and 2004 at University Hospital Marburg, were examined semiquantitatively for stromal content of CD34+ fibroblasts and α-SMA+ myofibroblasts. Partial preservation of CD34+ fibroblasts in the tumor stroma of ILC was confirmed. Absence of CD34+ fibroblasts in the tumor stroma significantly correlated with the presence of α-SMA+ myofibroblasts (p = 0.010), positive lymph node status (p = 0.004), and pN stage (p = 0.006). Stromal loss of CD34+ fibroblasts was significantly associated with lower overall and disease-free survival rates (p = 0.012 and 0.013, respectively). Multivariate analysis adjusted for pT and pN stage revealed stromal loss of CD34+ fibroblasts as independent prognostic parameter (p = 0.05). To our knowledge, this is the first report defining prognostically relevant stromal subtypes of ILC with long-term follow-up. Future research targeting the potential diagnostic and therapeutic implications of CD34+ fibroblasts and CAF in breast cancer, especially ILC, is a promising field of interest.

Actin isoform expression patterns in adult extracardiac and cardiac rhabdomyomas indicate a different cell of origin.

Rhabdomyomas are rare striated muscle-type tumors arising in the heart or in soft tissues. Using a monoclonal antibody specific for the cardiac isoform of α-actin (α-cardiac actin, α-CAA), differential expression patterns in striated muscle tissues were reported previously. The purpose of the present study was to determine whether the α-actin isoform specificity is maintained in rhabdomyomas according to their origin, comparing extracardiac to cardiac rhabdomyomas. We immunohistochemically investigated adult extracardiac (pharyngeal) rhabdomyomas (n = 4) and cardiac rhabdomyomas (n = 7) employing isoform-specific monoclonal antibodies. The extracardiac rhabdomyomas revealed only a few scattered α-CAA-positive tumor cells (antibody cAc1-20.42) while the cardiac rhabdomyomas exhibited abundant expression of α-CAA, indicating a close relatedness to cardiac muscle fibers. The α-skeletal actin (α-SKA) specific monoclonal antibody (3B3) produced the reverse results. General sarcomeric antibodies (HHF35 and Alpha Sr-1) displayed strong positivity in all rhabdomyomas studied. Alpha-smooth muscle actin (α-SMA) was negative or heterogeneously positive in extracardiac and cardiac rhabdomyomas. Our results suggest that despite similar morphology, the intrinsic differential alpha-actin isoform specificity of mature skeletal vs. cardiac muscle is maintained in extracardiac and cardiac rhabdomyomas. Thus, adult extracardiac rhabdomyomas differentiate towards mature skeletal muscle although they may exhibit centrally placed nuclei like cardiac muscle cells, while cardiac rhabdomyomas reflect true cardiac muscle differentiation. Our findings appear to indicate a different biological nature of cardiac and extracardiac rhabdomyomas, probably related to a different cell of origin. To our knowledge, this is the first report suggesting a derivation of extracardiac and cardiac rhabdomyomas from skeletal and cardiac muscle cells, respectively.

Perilipin 1 is a highly specific marker for adipocytic differentiation in sarcomas with intermediate sensitivity.

PURPOSE: Liposarcomas are the most common soft tissue sarcomas of adults. The identification of lipoblastic cells in soft tissue sarcomas is mandatory for the diagnosis of most subtypes of liposarcomas but may be difficult in conventional histology. The present study focuses on the expression and possible diagnostic impact of two PAT family proteins, perilipin 1/perilipin and perilipin 2/adipophilin in human liposarcomas. METHODS: Eighty-seven cases of liposarcomas and 30 cases of non-lipomatous sarcomas were investigated immunohistochemically for perilipin 1 and 2 using entire tissue sections. Statistical analyses were performed using appropriate tests. RESULTS: Most liposarcomas and non-lipomatous sarcomas displayed positivity for perilipin 2. In contrast, while more than two-thirds of liposarcomas presented perilipin 1 positivity, all non-lipomatous sarcomas studied were negative for this marker, with statistical significance (p < 0.001). Perilipin 1 expression increased with adipocytic differentiation of liposarcoma subtypes showing statistical significance (p < 0.001). Non-lipomatous sarcomas demonstrated variable expression levels of perilipin 2. The expression level of perilipin 2 appeared to be correlated with tumor cell degeneration, e.g., through hypoxia. CONCLUSIONS: Perilipin 2 is not well suitable for distinction between liposarcomas and non-lipomatous sarcomas. However, perilipin 1 appeared to be a highly specific marker for liposarcoma and adipocytic differentiation in sarcomas with intermediate sensitivity.

Osseous hemangioma of the seventh cervical vertebra with osteoid formation mimicking metastasis: a case report.

INTRODUCTION: We report the case of an osseous hemangioma located in the seventh cervical vertebra with reactive osteoid formation and non-typical findings in the radiological and the histopathological examination, mimicking metastasis of a malignant tumor. To our knowledge, this is the first description of such a case in the literature. CASE PRESENTATION: A 44-year-old otherwise healthy Caucasian German woman presented with a discrete sensorimotor loss of both upper limbs. Radiologically, an osteolysis in the seventh cervical vertebra suggestive of metastasis of a malignant neoplasm was diagnosed. After performing corporectomy and cage implantation of C7 on the patient, the histopathological examination was complicated by marked osteoid formation obscuring the true diagnosis of an osseous hemangioma with reactive osteoid formation. CONCLUSION: Though hemangioma of the bone is a rare tumorous lesion in the cervical spine, it has to be taken into consideration as a reason for neck pain and sensomotoric loss of the upper limbs. Atypical radiological and histopathological presentations may hinder determination of the correct diagnosis. The treatment of such lesions must follow clinical guidelines but may be difficult to define in some cases when the correct diagnosis is not known at the time when therapy starts.