Management of Poorly Differentiated Thyroid Cancer and Differentiated High-Grade Thyroid Carcinoma.

Thyroid carcinoma of follicular cell origin exists on a histopathologic and clinical spectrum. The authors focus on the category of tumors that fall between the very favorable well-differentiated thyroid carcinomas and the very unfavorable anaplastic thyroid carcinomas. These intermediately aggressive tumors include poorly differentiated thyroid carcinoma and the newly defined differentiated high-grade thyroid carcinoma. Both diagnoses require certain histopathologic requirements be met in order to accurately identify these tumors post-operatively. Management remains primarily surgical though adjunctive treatments such as molecular targeted therapies (eg, tyrosine kinase inhibitors) and differentiation therapy (to restore tumor response to radioactive iodine) are also becoming available.

A Comprehensive Review and Insights into the New Entity of Differentiated High-Grade Thyroid Carcinoma.

Differentiated high-grade thyroid carcinoma (DHGTC) is a new subset within the spectrum of thyroid malignancies. This review aims to provide a comprehensive overview of DHGTC, focusing on its historical perspective, diagnosis, clinical characteristics, molecular profiles, management, and prognosis. DHGTC demonstrates an intermediate prognosis that falls between well-differentiated thyroid carcinoma and anaplastic thyroid carcinoma. Previously unenumerated, this entity is now recognized for its significant impact. Patients with DHGTC often present at an older age with advanced disease and exhibit aggressive clinical behavior. Molecularly, DHGTC shares similarities with other thyroid malignancies, harboring driver mutations such as BRAFV600E and RAS, along with additional late mutations. The unique behavior and histologic features of DHGTC underscore the necessity of precise classification for prognostication and treatment selection. This highlights the critical importance of accurate diagnosis and recognition by pathologists to enrich future research on this entity further.

Oral Papillary Squamous Cell Carcinoma and Oral Squamous Cell Carcinoma: A Histopathological and Immunohistochemical Comparative Study.

PURPOSE: The aim of the study is to investigate the immunohistochemical expression of both Alpha smooth muscle actin and Transforming Growth Factor beta and compare their expression in oral papillary squamous cell carcinoma with their expression in different histological grades of oral squamous cell carcinoma. A correlation between these immuno-histochemical expressions and histological findings will then be performed. The research question is "Do the percentages of α-SMA and TGF-ß immune-expression in OPSCC differ from that in the conventional OSCC?". METHODS: This will be achieved by collecting archival blocks of oral papillary squamous cell carcinoma and different grades of oral squamous cell carcinoma, staining the specimens with Transforming Growth Factor beta and alpha smooth muscle actin, then measuring the mean staining index of expression in each group and the area percent of both markers. RESULTS: Results revealed that transforming growth factor beta expression in the epithelium was high in all cases of well-differentiated squamous cell carcinoma, most oral papillary squamous cell carcinoma, and poorly differentiated oral squamous cell carcinoma. On the other hand, different grades of oral squamous cell carcinoma showed a high staining index of alpha smooth muscle actin expression in the stroma. While cases of oral papillary squamous cell carcinoma were either moderate or low-staining. CONCLUSIONS: Oral papillary squamous cell carcinoma has a favourable prognosis compared to different histological grades, and the prognosis does not depend only on histological grade but also on other prognostic factors.

Primary Neuroendocrine Tumor of the Testis Associated With Cardiac Symptoms: A Case Report and Literature Review.

Well-differentiated neuroendocrine tumors of the testis are exceedingly rare. Here, we report the case of a 47-year-old male patient complaining of cardiac symptoms with a right testicular mass. A right radical orchiectomy was performed. The histopathological findings showed a well-differentiated neuroendocrine tumor with positive synaptophysin and chromogranin A immunostains.

The significance of BAP1 and MTAP/CDKN2A expression in well-differentiated papillary mesothelial tumour: a series of 21 cases and a review of the literature.

The nomenclature and diagnostic criteria of well-differentiated papillary mesothelial tumour (WDPMT) have been changed in the 2021 World Health Organization (WHO) classification of thoracic tumours, and a new entity, mesothelioma in situ (MIS), introduced. Histologically these two entities may be similar. However, MIS is regarded as a precursor to invasive mesothelioma and requires demonstration of loss of BAP1 and/or MTAP/CDKN2A for diagnosis, whereas performance of these ancillary tests is desirable but not essential for a diagnosis of WDPMT, in which the significance of BAP1 and/or MTAP/CDKN2A loss is not well understood or well defined. Against this backdrop, we undertook an investigation of 21 cases of WDPMT, identified from our case files and diagnosed according to 2021 WHO criteria, to explore the relationship between histology and BAP1 and MTAP/CDKN2A expression with clinical features including asbestos exposure, focality of tumours and clinical outcome. There were 18 women and three men, with ages ranging from 23-77 years (median 62 years), in which six had a history of asbestos exposure, two had no exposure, and in 13 exposure history was unavailable. Of 20 peritoneal tumours and one pleural tumour, 13 were detected incidentally at the time of surgery for unrelated conditions and eight peritoneal tumours were multifocal at the time of diagnosis. BAP1 immunohistochemistry (IHC) was performed in all 21 tumours, with nine tumours showing BAP1 expression loss. MTAP/CDKN2A testing was performed in 14 tumours, comprising MTAP IHC in 12 and CDKN2A fluorescence in situ hybridisation (FISH) in two, with three tumours showing MTAP/CDKN2A expression loss. Two tumours with MTAP/CDKN2A loss also showed BAP1 expression loss. Four patients progressed to invasive mesothelioma, including one male with a pleural tumour and asbestos exposure, and three females with multifocal peritoneal tumours, two with asbestos exposure and one without exposure. BAP1 expression loss was seen in all tumours from the four patients who progressed to invasive mesothelioma, whilst two of these tumours showed retained MTAP IHC and two were not tested. There was one patient with a tumour with MTAP loss and retained BAP1 who died from unrelated causes 5 months after diagnosis. Eight patients received WDPMT-specific treatment in addition to the initial excision. Survival for all patients ranged from 4-218 months, with one patient dying of mesothelioma at 49 months. Based on our results in this series of 21 patients with WDPMT diagnosed according to 2021 WHO criteria, we propose that WDPMT with BAP1 expression loss may best be regarded as papillary MIS and that a history of asbestos exposure and the presence of multifocal tumours in patients diagnosed with WDPMT should prompt ancillary testing with BAP1 IHC. Further we propose that BAP1 IHC should be essential in the diagnosis of WDPMT, with the diagnosis restricted to those tumours which show retained BAP1 expression. However more studies in larger cohorts of patients are needed to explore the relationship between BAP1 expression and MTAP loss in WDPMT, which will help to define this entity and separate it more clearly from MIS and invasive mesothelioma.

Expression Profiling of EMT Transcriptional Regulators ZEB1 and ZEB2 in Different Histopathological Grades of Oral Squamous Cell Carcinoma Patients.

Background: Pakistan has a high burden of oral cancers, with a prevalence rate of around 9%. Oral Squamous Cell Carcinoma (OSCC) accounts for about 90% of oral cancer cases. Epithelial to Mesenchymal Transition (EMT) gets highly stimulated in tumor cells by adopting subsequent malignant features of highly invasive cancer populations. Zinc Finger E-Box binding factors, ZEB1 and ZEB2, are regulatory proteins that promote EMT by suppressing the adherent ability of cells transforming into highly motile cancerous cells. The present study aimed to analyze the expression of EMT regulators, ZEB1 and ZEB2, and their association with the clinicopathological features in different grades of OSCC patients. Methods: Tissue samples were collected for both case and control groups from the recruited study participants. Cancer tissues (cases) were collected from the confirmed OSCC patients, and healthy tissues (controls) were collected from third-molar dental extraction patients. The study participants were recruited with informed consent and brief demographic and clinical characteristics. The case group was further segregated with respect to the histological cancer grading system into well-differentiated (WD), moderately differentiated (MD), and poorly differentiated (PD) squamous cell carcinoma (SCC) groups. RNA was extracted from the tissue samples for expression profiling of ZEB1 and ZEB2 genes through quantitative real-time PCR (qRT-PCR). Results: All of the recruited participants had a mean age of 46.55 ± 11.7 (years), with most of them belonging to Urdu speaking ethnic group and were married. The BMI (kg/m2) of the healthy participants was in the normal range (18-22 kg/m2). However, BMI was found to be reduced with the proliferation in the pathological state of cancer. The oral hygiene of patients was better than the healthy participants, possibly due to the strict oral hygiene practice concerns of consultants. Every recruited OSCC patient had one or multiple addiction habits for more than a year. Patients reported health frailty (46.6%), unhealed mouth sores (40%), swallowing difficulties and white/reddish marks (80%), and restricted mouth opening (64.4%). Furthermore, 82.2% of the recruited patients observed symptoms within 1-12 months, and buccal mucosa was the most exposed tumor site among 55.6% of the patients. Expression profiling of EMT regulators showed gradual over-expressions of ZEB1 (8, 20, and 42 folds) and ZEB2 (4, 10, and 18 folds) in respective histological cancer grades. Conclusion: High expressions of ZEBs have been significantly associated with cancer progression and poor health. However, no association was found between OSCC with other clinicopathological features when compared to healthy controls.

Endobronchial Obstruction: A Case of Well-Differentiated Liposarcoma.

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) is a rare subtype of endobronchial tumor that has been rarely reported in medical literature. Due to its low incidence, distinguishing it from endobronchial lipoma poses a significant diagnostic challenge, necessitating histopathologic and cytogenetic analysis. As of today, the treatment and surveillance protocols for these neoplasms remain poorly defined, often resulting in their misclassification and treatment as endobronchial lipomas. We present a case involving a 72-year-old male who presented with worsening dyspnea and cough. Diagnostic imaging revealed an endobronchial lesion obstructing the left main bronchus. The patient underwent a flexible bronchoscopy that identified a polypoid mass causing significant obstruction, which was subsequently resected via cryoablation. Histopathology confirmed ALT/WDL, supported by genetic analysis revealing chromosomal alterations. Following the intervention, symptoms resolved, with no recurrence on follow-up imaging. Differentiating ALT/WDL from endobronchial lipomas is necessary not only because it influences treatment decisions but also because it can significantly affect the prognosis of patients diagnosed with ALT/WDL. In this case, we emphasize the importance of considering ALT/WDL in the differential diagnosis of endobronchial tumors and highlight the use of flexible bronchoscopy as a viable substitute for rigid bronchoscopy, serving not only as a diagnostic tool but also as a therapeutic method.

Case report: A rare case of very well-differentiated gastric adenocarcinoma of gastric type with a lymphovascular invasion.

Background: Very well-differentiated gastric adenocarcinoma (VWDA) is a rare variant of gastric cancer, for which the diagnostic criteria and clinical behavior are not fully established. We reported a case of an intramucosal VWDA of gastric type with a lymphovascular invasion (LVI). Case presentation: A 67-year-old female was diagnosed as intramucosal gastric adenocarcinoma after a biopsy at the local hospital 3 weeks ago and then visited our hospital for further treatment. The endoscopic examination in our hospital showed a rough, slightly faded, 30-mm, flat, and elevated lesion on the lesser curvature of the middle gastric body. Histopathologically, the lesion consisted of superficial foveolar-type papillary adenocarcinoma and deep pyloric gland-type tubular adenocarcinoma. The immunostaining results showed that the foveolar-type papillary adenocarcinoma was positive for MUC5AC and had a high index of Ki-67, but the pyloric gland-type tubular adenocarcinoma was positive for MUC6 and had a low index of Ki-67. Both components were negative for MSH2 and MSH6, which suggested the high microsatellite instability phenotype. Moreover, a LVI was detected in the lesion. The pathological diagnosis was VWDA of gastric type. Conclusion: The case has unique histological and immunophenotypic characteristics, which not only indicates the importance of architectural features in the diagnosis of VWDA but also further proves that the aggressive behavior of VWDA is correlated with tumor histological type and immunophenotype.

Rare Nasopharyngeal Neuroendocrine Tumor in a 26-Year-Old Female: Case Report and Literature Review.

Neuroendocrine neoplasms (NENs) represent a collection of highly varied tumors that originate from neuroendocrine cells. They are considered rare tumors that predominantly affect the lungs. Epithelial NENs can be categorized into neuroendocrine tumors (NETs) and neuroendocrine carcinomas. It is extremely rare for NET grade 1 (NET G1) to exist in the nasopharynx, these tumors are slow-growing and the onset of symptoms and identification of the tumor may take several years. The majority of cases occur in ages between 60 and 65 years. In this article, we present a case of a 26-year-old female who presented with recurrent epistaxis and nasal obstruction for one and a half years. Magnetic resonance imaging revealed a substantial nasopharyngeal mass. Subsequently, a biopsy was conducted, and the histopathological results indicated a NET G1. Our literature review revealed 5 cases of NET G1 in the nasopharynx, with our patient being the youngest among all published cases.

Sclerosing well-differentiated liposarcoma: two diagnostically challenging mimicker cases and a literature review.

Liposarcoma is a malignant soft tissue tumor with several subtypes, the most common of which is well-differentiated liposarcoma (WDL) or atypical lipomatous tumor (ALT). WDL/ALTs are further divided into three histological subtypes, including lipoma-like, sclerosing, and inflammatory. While the majority of these tumors are predominantly fatty, the sclerosing variant demonstrates diverse histologic and radiographic characteristics, including variable amounts of fibrosis and fat. Because of this histological variability and relative rarity, the sclerosing WDL/ALT can present diagnostic dilemmas. We present two cases of sclerosing WDL/ALT, both of which demonstrated high degrees of fibrosis and a paucity of fat, mimicking desmoid fibromatosis and other fibrotic soft tissue tumors. Thus, it is important for radiologists to be aware of the subtypes of liposarcoma and their unique characteristics, and to consider sclerosing WDL/ALT in cases of fibrotic soft tissue tumors.

Parosteal osteosarcoma with low grade chondrosarcoma and liposarcoma components. A rare histologic variant. Case report and literature review.

INTRODUCTION: conventional parosteal osteosarcoma is an uncommon malignant bone tumor, comprising 4% of all osteosarcomas. Although rare, parosteal osteosarcoma is the most common type of osteosarcoma of the bone surface. We present the clinical, histological and imaging characteristics of a rare histologic variant of a parosteal osteosarcoma, review the literature and emphasize the importance of radio-pathological correlation as well as the interpretation of a representative biopsy in order to obtain the correct diagnosis. CASE REPORT: a 36-year old woman began her condition one year prior to admission to the hospital with increased volume in the left knee and pain. Image studies showed a juxtacortical heterogeneous tumor localized on the posterior surface of the distal femoral metaphysis. An incisional biopsy was performed, with the diagnosis of a Parosteal Osteosarcoma and a wide surgical resection was undertaken. According to the findings of the surgical specimen, the diagnosis of a Parosteal Osteosarcoma with low grade chondrosarcoma and liposarcoma components was made. The knowledge of this rare parosteal osteosarcoma variant can lead the orthopedic oncologists to avoid overlooking the adipose component and provide adequate surgical margins. CONCLUSION: we present the clinical, histological and imaging characteristics of a Parosteal Osteosarcoma with low grade liposarcoma and chondrosarcoma components.

INTRODUCCIÓN: el osteosarcoma parosteal convencional es un tumor óseo maligno poco común, que comprende el 4% de todos los osteosarcomas. Aunque es poco común, el osteosarcoma parosteal es el tipo más común de osteosarcoma de la superficie ósea. Presentamos las características clínicas, histológicas y de imagen de una variante histológica rara de un osteosarcoma parosteal, revisamos la literatura y enfatizamos la importancia de la correlación radio-patológica, así como la interpretación de una biopsia representativa para obtener el diagnóstico correcto. REPORTE DE CASO: mujer de 36 años inició su cuadro un año antes de su ingreso al hospital con aumento de volumen en rodilla izquierda y dolor. Los estudios de imagen mostraron una tumoración heterogénea yuxtacortical localizada en la superficie posterior de la metáfisis femoral distal. Se realizó biopsia incisional, con diagnóstico de osteosarcoma parosteal y se realizó resección quirúrgica amplia. De acuerdo con los hallazgos de la pieza quirúrgica se realizó el diagnóstico de osteosarcoma parosteal con componentes de condrosarcoma y liposarcoma de bajo grado. El conocimiento de esta rara variante de osteosarcoma parosteal puede llevar a los ortopedistas oncólogos a considerar otros componentes y proporcionar márgenes quirúrgicos adecuados. CONCLUSIÓN: presentamos las características clínicas, histológicas y de imagen de un osteosarcoma parosteal con componentes de liposarcoma y condrosarcoma de bajo grado.

MRI Differential Diagnosis and Guidance for Puncture Biopsy of Musculoskeletal Dedifferentiated Liposarcoma and Well Differentiated Liposarcoma.

Objective: The study aimed to investigate the significantly different imaging characteristics of musculoskeletal dedifferentiated liposarcoma (DDLP) and well differentiated liposarcoma (WDLP) on MRI, which in turn could guide puncture biopsy. Materials and Methods: This study included 14 patients with DDLP and 16 patients with WDLP, all of whom were confirmed by histopathological examination. The MRI manifestations of these two pathologies were retrospectively reviewed and compared. Furthermore, a step-by-step procedure regarding preoperative puncture biopsy of fatty masses that are suspicious for WD/DD was designed. Results: Fatty signals can be found in almost all WDs, with a greater proportion of non-fatty areas in DD compared to WD, and it is reasonable to consider WD more likely when the non-fatty areas of the tumor are <25% (p < 0.05), while it is reasonable to consider DD more likely when the non-fatty areas of the tumor are >50% (p < 0.05), and the MRI signals in DD are more complex, inhomogeneous (p < 0.01), usually showed significant enhancement (p < 0.01), and the margins of the tumor were usually indistinct (p < 0.01); and imaging features such as tumor size, vascularity, necrosis, and peritumoral edema did not serve as distinguishing features between the two (p > 0.05). Conclusion: DD has a greater proportion of non-fatty components, with more complex and inhomogeneous MRI signals, and typically shows significant enhancement, with usually indistinct margins of the tumor, in which the inhomogeneous manifestations are associated with the histological components. The possibility of DD should be considered in fatty tumors with non-fatty areas > 25%, for which puncture biopsy is necessary, while simultaneous puncture of low, moderate, high-signal areas within the non-fatty area could improve the accuracy of preoperative puncture pathology.

A Case Report of a 17 cm Retropharyngeal Well-Differentiated Liposarcoma With Bilateral Neck Extension.

Liposarcomas, malignant adipocytic tumors, primarily manifest in the lower extremities and retroperitoneum, with a strikingly low incidence in the head and neck region. Symptomatology typically remains absent until the tumor attains significant size, leading to cosmetic concerns or compression-related complications. This report presents a unique case of well-differentiated retropharyngeal liposarcoma in an 81-year-old male, emphasizing diagnostic challenges, management strategies, and the crucial role of Mouse double minute 2 (MDM2) fluorescence in situ hybridization (FISH) analysis in confirmation. The patient exhibited dysphagia attributed to a retropharyngeal mass, prompting suspicion of malignancy. Diagnostic assessments, including flexible laryngoscopy and percutaneous tru-cut biopsy, highlighted unique features such as atypical nuclear features in adipocytes. MDM2 FISH analysis definitively confirmed the diagnosis by detecting MDM2 gene amplification. The rarity of retropharyngeal liposarcomas complicates diagnosis, often leading to confusion with benign lesions. Surgical excision, the mainstay of treatment, varies based on tumor size and extension. In this case, a left neck dissection via a hockey stick incision successfully resected a 17 cm well-differentiated liposarcoma. Pathologic analysis revealed focal involvement of resection margins, with no complications or vocal cord damage. In conclusion, retropharyngeal liposarcomas pose diagnostic challenges, warranting reliance on MDM2 FISH analysis for accurate confirmation. Early surgical intervention, guided by tumor size and extension, is paramount for optimal outcomes in managing these rare tumors. This case underscores the significance of a detailed surgical approach in achieving successful outcomes for retropharyngeal liposarcomas.

Pleomorphic Lipoma of the Parotid Gland: A Potential Cytological Pitfall.

Pleomorphic lipoma is an uncommon benign adipocytic tumor that arises from the subcutis. It has no risk of recurrence, metastasis or malignant transformation. The cytological findings of pleomorphic lipoma are often overdiagnosed as suspicious of malignancy owing to the pleomorphic morphology of the floret giant cells. We present a rare case of pleomorphic lipoma of the parotid gland which was misdiagnosed as a malignant tumor on fine needle aspiration cytology. Histopathological examination and positive expression for CD34 helped to arrive at the final diagnosis. Awareness about this rare entity will prevent overdiagnosis as a malignant tumor and unnecessary radical resections for this benign tumor.

INSM1 is a useful neuroendocrine marker to differentiate pancreatic serous cystadenoma from pancreatic well-differentiated neuroendocrine tumors in cytology and surgical specimens.

INTRODUCTION: Differentiating pancreatic serous cystadenoma (SCA) from well-differentiated neuroendocrine tumors (WDNETs) based on histomorphology is critical yet challenging, particularly in small biopsy samples. Our study aimed to examine the expression profile of INSM1 in cytologic and surgical resection specimens from pancreatic SCA to evaluate its potential as a discriminative marker against pancreatic WDNET. METHODS: We characterized INSM1 immunohistochemistry in 34 patients with pancreatic SCA, comprising 23 surgical resections and 11 cytology specimens. As a control, we used 28 cytology specimens from pancreatic WDNET. Clinical information was retrieved through a review of electronic medical records. RESULTS: All 11 pancreatic SCA cytology specimens and 15 of 23 pancreatic SCA surgical resections exhibited absent INSM1 immunostaining. Each of the remaining eight surgical resection specimens demonstrated 1 % immunoreactivity. In contrast, 27 out of 28 (96 %) pancreatic WDNET cytology specimens were positive for INSM1 immunostaining, with a median immunoreactivity of 90 % and a range of 30-90 %. Overall, INSM1 immunostains perform similarly to chromogranin and synaptophysin in pancreatic SCA. CONCLUSIONS: The results indicate that INSM1 immunohistochemistry staining may serve as a useful neuroendocrine marker to differentiate pancreatic SCA from pancreatic WDNET in clinical practice. To our knowledge, this represents the first large-scale study to evaluate INSM1 immunostaining in surgical and cytology specimens from pancreatic SCA.

GLI1 Coamplification in Well-Differentiated/Dedifferentiated Liposarcomas: Clinicopathologic and Molecular Analysis of 92 Cases.

GLI1(12q13.3) amplification is identified in a subset of mesenchymal neoplasms with a distinct nested round cell/epithelioid phenotype. MDM2 and CDK4 genes are situated along the oncogenic 12q13-15 segment, amplification of which defines well-differentiated liposarcoma (WDLPS)/dedifferentiated liposarcoma (DDLPS). The 12q amplicon can occasionally include GLI1, a gene in close proximity to CDK4. We hereby describe the first cohort of GLI1/MDM2/CDK4 coamplified WD/DDLPS. The departmental database was queried retrospectively for all cases of WD/DDLPS having undergone next-generation (MSK-IMPACT) sequencing with confirmed MDM2, CDK4, and GLI1 coamplification. Clinicopathologic data was obtained from a review of the medical chart and available histologic material. Four hundred eighty-six WD/DDLPS cases underwent DNA sequencing, 92 (19%) of which harbored amplification of the GLI1 locus in addition to that of MDM2 and CDK4. These included primary tumors (n = 60), local recurrences (n = 29), and metastases (n = 3). Primary tumors were most frequently retroperitoneal (47/60, 78%), mediastinal (4/60, 7%), and paratesticular (3/60, 5%). Average age was 63 years, with a male:female ratio of 3:2. The cohort was comprised of DDLPS (86/92 [93%], 6 of which were WDLPS with early dedifferentiation) and WDLPS without any longitudinal evidence of dedifferentiation (6/92, 7%). One-fifth (13/86, 17%) of DDLPS cases showed no evidence of a well-differentiated component in any of the primary, recurrent, or metastatic specimens. Dedifferentiated areas mostly showed high-grade undifferentiated pleomorphic sarcoma-like (26/86,30%) and high-grade myxofibrosarcoma-like (13/86,16%) morphologies. A disproportionately increased incidence of meningothelial whorls with/without osseous metaplasia was observed as the predominant pattern in 16/86 (19%) cases, and GLI1-altered morphology as described was identified in a total of 10/86 (12%) tumors. JUN (1p32.1), also implicated in the pathogenesis of WD/DDLPS, was coamplified with all 3 of MDM2, CDK4, and GLI1 in 7/91 (8%) cases. Additional loci along chromosomal arms 1p and 6q, including TNFAIP3, LATS1, and ESR1, were also amplified in a subset of cases. In this large-scale cohort of GLI1 coamplified WD/DDLPS, we elucidate uniquely recurrent features including meningothelial whorl-like and GLI-altered morphology in dedifferentiated areas. Assessment of tumor location (retroperitoneal or mediastinal), identification of a well-differentiated liposarcoma component, and coamplification of other spatially discrete genomic segments (1p and 6q) might aid in distinction from tumors with true driver GLI1 alterations.

A case report of typical well-differentiated papillary mesothelial tumor diagnosed by internal thoracoscopy and literature review.

We report a case of well-differentiated papillary mesothelial tumor (WDPMT) diagnosed using internal thoracoscopic biopsy in a patient who has suffered from recurrent pleural effusions for over 35 years together with a history of elevated CA125. We hope to provide a case for the diagnosis of this rare benign and preinvasive pleural tumor and recommend that internal thoracoscopy may be a good choice in these recurrent pleural effusion patients especially for those minimal lesions not easily detected using CT scan.

Well-differentiated liposarcomas and dedifferentiated liposarcomas: Systemic treatment options for two sibling neoplasms.

Well-differentiated liposarcomas (WDLPS) and dedifferentiated liposarcomas (DDLPS) account for 60 % of all liposarcomas, reflecting the heterogeneity of this type of sarcoma. Genetically, both types of liposarcomas are characterized by the amplification of MDM2 and CDK4 genes, which indicates an important molecular event with diagnostic and therapeutic relevance. In both localized WDLPS and DDLPS of the retroperitoneum and the extremities, between 25 % and 30 % of patients have local or distant recurrence, even when perioperatively treated, with clear margins present. The systemic treatment of WDLPS and DDLPS remains a challenge, with anthracyclines as the gold standard for first-line treatment. Several regimens have been tested with modest results regarding their efficacy. Herein we discuss the systemic treatment options for WDLPS and DDLPS and review their reported clinical efficacy results.

Surgical Management of a Large Well-Differentiated Liposarcoma in the Supraclavicular Fossa: A Case Report.

Background: Liposarcomas originating in the supraclavicular fossa are exceptionally rare, with only a few documented cases in the medical literature. Methods: We present a unique case involving a remarkably large indolent, atypical lipomatous tumor or well-differentiated liposarcoma situated in the right supraclavicular region, measuring 18 cm × 18 cm × 17 cm. To our knowledge, this represents the largest liposarcoma found in the supraclavicular fossa reported in the literature. This unique case highlights the effective management of a remarkably large supraclavicular liposarcoma through complete surgical resection. Result and Conclusion: Our findings further support the existing consensus that complete surgical removal is often curative for well-differentiated liposarcoma.