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Background: Pancreatic cancer is among the malignancies with the poorest prognosis, largely due to its aggressive nature and resistance to conventional therapies. Case Summary: This report describes the case of a 69-year-old male patient with stage IV primary lung adenocarcinoma presenting with high levels of programmed death-ligand 1 (PD-L1). Simultaneously, abdominal computed tomography (CT) showed a dilated pancreatic duct at the level of the pancreatic head and a hypodense lesion in the uncinate process involving the superior mesenteric artery. Fine-needle aspiration (FNA) of the pancreatic lesions was negative. After three cycles of chemoimmunotherapy, positron emission tomography–computed tomography (PET-CT) showed complete remission of the lung nodules, lymphadenopathy, and pleural thickening, as well as a decrease in the size of the pancreatic lesion. After another six months, a PET-CT scan showed a focal increased uptake in the pancreatic mass in the same location, indicating disease progression. A core biopsy of the pancreatic tumor showed atypical spindle cell morphology with positive staining for vimentin, characteristic of mesenchymal differentiation with no apparent epithelial features. Comprehensive molecular profiling through Caris Molecular Intelligence® revealed four genes with actionable mutations in the pancreatic tissue, including KRAS (p.G12D) and TP53 (p.R175H). These molecular findings suggested the diagnoses of sarcomatoid carcinoma and conventional pancreatic ductal adenocarcinoma with epithelial–mesenchymal transition. Primary mesenchymal tumors and neuroendocrine neoplasms were excluded because immunohistochemistry was negative for anaplastic lymphoma kinase (ALK), smooth muscle actin (SMA), desmin, CD34, signal transducer and activator of transcription 6 (STAT6), S100, HMB45, CD117, discovered on GIST-1 (DOG1), CD56, progesterone, and synaptophysin. However, despite multiple rounds of systemic chemotherapy, immunotherapy, and radiation, his pancreatic disease rapidly deteriorated and metastasized to the liver and bone. Conclusions: Despite multiple lines of treatment, the patient's condition worsened and he succumbed to his pancreatic malignancy. This study highlights the clinical characteristics, diagnosis, and treatment of rare pancreatic cancer, emphasizing the importance of molecular testing and histopathological biomarkers in personalizing treatment. It also provides insights into promising therapeutic approaches for similar cases with an unusual presentation. [ABSTRACT FROM AUTHOR]

ABSTRACT Background Method Results Conclusion Melanocytic tumors with perineuriomatous differentiation may pose diagnostic challenges. This study explores characteristics of perineuriomatous melanocytic nevi, an entity merging features of perineurioma and melanocytic nevi. The aim is to elucidate histopathological features of perineuriomatous nevi to allow dermatopathologists to recognize them and differentiate them from other spindle cell lesions.This study reviews four cases (2020–2023) of melanocytic nevi with perineuriomatous components.All cases comprised adults (median age: 64.50, mean age: 60.25), with a female predominance, and exhibited clinical features characterized by irregular brown macules or papules on the trunk and extremities. Histopathologically, all lesions were compound, and biphasic patterns were evident, encompassing superficial nevoid and deeper spindled populations arranged in whorled fascicles and embedded in a sclerotic or myxoid stroma. Immunohistochemistry revealed expression of at least one perineuriomatous marker in deeper cells. Cases were assessed and compared with previously published cases for comprehensive insights. Three of our four cases demonstrated the presence of a junctional component that was smaller than the dermal component. We suggest that this unusual feature, which we term the “reverse shoulder”, may allow dermatopathologists to help consider perineurial differentiation in the appropriate setting.Perineuriomatous nevi can pose diagnostic challenges. This study contributes to the growing body of literature on perineuriomatous nevi, emphasizing their unique features and the importance of accurate diagnosis to avoid unnecessary interventions. [ABSTRACT FROM AUTHOR]

Giant Cell Tumour of soft-tissue (GCT-ST) is a rare neoplasm which is morphologically similar and genetically unrelated to GCT bone and included under intermediate rarely metastasising category. They are reported predominantly in superficial soft-tissue of the upper and lower extremities and less frequently in head and neck. The authors present a case of malignancy arising from GCT-ST involving thyroid gland mimicking primary thyroid malignancy. An 80-year-old female presented in the Outpatient Department (OPD) with a neck swelling of six months duration and sudden increase in size and hoarseness of voice for two weeks. An upfront surgery was planned with a clinical suspicion of primary thyroid neoplasm. Intraoperatively, the thyroid gland was replaced by multiple nodules, which were seen infiltrating adjacent structures. Due to the invasive nature, only a subtotal thyroid resection was done. The histopathology showed predominantly neoplasm composed of mononuclear cells and sheets of osteoclast like giant cells with foci showing features of malignancy. Histomorphology along with Immunohistochemistry (IHC) confirmed a diagnosis of GCT-ST involving the thyroid gland. Due to the rapidly progressive nature of the disease, the patient succumbed within two months.

The previous endometrial cancer (EC) FIGO staging primarily relied on the extent of the disease spread into the anatomical sites at diagnosis. The most recent one (2023) incorporates clinicopathological features such as histological subtype, tumor grade, the extent of lymphovascular space invasion (LVI), and, when available, molecular subtypes of EC. The emphasis on accurate histological typing, tumor grading, and the molecular features of the cancer is stronger than ever. This review addresses challenging diagnostic patterns in the histologic subtyping and grading EC under five categories: 1. EC with spindle cells, 2. EC with clear cells, 3. EC with a papillary architecture, 4. EC with a biphasic morphology, and 5. EC with a microglandular architecture. The morphological features differentiating low- and high-grade cancers are discussed, along with relevant clinical work-ups. Recent molecular genetic findings regarding the diagnosis and prognosis of the disease and the results of related clinical trials are summarized. The potential challenges in the evaluation of LVI follow these sections. The final section of the review includes an overview of the literature on incorporating molecular subtypes of EC into clinical practice. [ABSTRACT FROM AUTHOR]

Giant Cell Tumour of soft-tissue (GCT-ST) is a rare neoplasm which is morphologically similar and genetically unrelated to GCT bone and included under intermediate rarely metastasising category. They are reported predominantly in superficial soft-tissue of the upper and lower extremities and less frequently in head and neck. The authors present a case of malignancy arising from GCT-ST involving thyroid gland mimicking primary thyroid malignancy. An 80-year-old female presented in the Outpatient Department (OPD) with a neck swelling of six months duration and sudden increase in size and hoarseness of voice for two weeks. An upfront surgery was planned with a clinical suspicion of primary thyroid neoplasm. Intraoperatively, the thyroid gland was replaced by multiple nodules, which were seen infiltrating adjacent structures. Due to the invasive nature, only a subtotal thyroid resection was done. The histopathology showed predominantly neoplasm composed of mononuclear cells and sheets of osteoclast like giant cells with foci showing features of malignancy. Histomorphology along with Immunohistochemistry (IHC) confirmed a diagnosis of GCT-ST involving the thyroid gland. Due to the rapidly progressive nature of the disease, the patient succumbed within two months. [ABSTRACT FROM AUTHOR]

Objective: Primary Spindle cell tumors of the thyroid are very rare and comprise a myriad of differential diagnoses ranging from reactive proliferations to neoplastic processes. Differentiation amongst these rare entities is of utmost importance as it defines the prognosis and also guides future therapy. Case Report: Hereby, we present a rare case of spindle cell variant of papillary thyroid carcinoma in a sixty-four-year-old female which posed a diagnostic challenge on histology. Microscopy showed a well-circumscribed spindle cell lesion with areas of infiltration and entrapment of colloid-filled thyroid follicles. There were no characteristic features of papillary carcinoma. A panel of immunohistochemistry markers was advised which clinched the diagnosis as the spindle tumor cells were positive for thyroid papillary carcinoma markers. Although papillary carcinoma usually has an indolent course, however interestingly this patient presented with lymph node metastasis one month after surgery which cytologically showed features of malignant spindle cell neoplasm. Conclusion: Through this case report we wish to highlight the cytological, histopathological as well as immunohistochemical features of this rare variant of papillary carcinoma. [ABSTRACT FROM AUTHOR]

Abstract Background The primary pathological alterations of Pendred syndrome are endolymphatic pH acidification and luminal enlargement of the inner ear. However, the molecular contributions of specific cell types remain poorly characterized. Therefore, we aimed to identify pH regulators in pendrin-expressing cells that may contribute to the homeostasis of endolymph pH and define the cellular pathogenic mechanisms that contribute to the dysregulation of cochlear endolymph pH in Slc26a4 −/− mice. Methods We used single-cell RNA sequencing to identify both Slc26a4-expressing cells and Kcnj10-expressing cells in wild-type (WT, Slc26a4 +/+) and Slc26a4 −/− mice. Bioinformatic analysis of expression data confirmed marker genes defining the different cell types of the stria vascularis. In addition, specific findings were confirmed at the protein level by immunofluorescence. Results We found that spindle cells, which express pendrin, contain extrinsic cellular components, a factor that enables cell-to-cell communication. In addition, the gene expression profile informed the pH of the spindle cells. Compared to WT, the transcriptional profiles in Slc26a4 −/− mice showed downregulation of extracellular exosome-related genes in spindle cells. Immunofluorescence studies in spindle cells of Slc26a4 −/− mice validated the increased expression of the exosome-related protein, annexin A1, and the clathrin-mediated endocytosis-related protein, adaptor protein 2. Conclusion Overall, cell isolation of stria vascularis from WT and Slc26a4 −/− samples combined with cell type-specific transcriptomic analyses revealed pH-dependent alternations in spindle cells and intermediate cells, inspiring further studies into the dysfunctional role of stria vascularis cells in SLC26A4-related hearing loss.

Primary carcinomas of the lung are vastly represented by the conventional types of adenocarcinomas or squamous cell carcinomas. However, there are other types of non-small cell carcinomas that although uncommon represent a meaningful group that often pose a problem not only in diagnosis but also in classification. Spindle cell and/or giant cell carcinomas, although uncommon represent an important group of primary lung carcinomas. Important to highlight is that current criteria are rather ambiguous and likely not up to date, which renders the classification of these tumors somewhat more obscure. In addition, with the daily use of immunohistochemical stains, the classification of these tumors may also pose a different problem in the proper allocation of these tumors. Proper classification is highly important in the selection process that takes place using such material for molecular analysis. The current molecular characteristics of these tumors are limited and lack more in-depth studies and analyses that can provide specific targets for the treatment of patients with these tumors. The current review attempts to highlight the shortcomings in the current classification and definitions of these neoplasms as well as the more current view regarding these tumors when the use of immunohistochemical stains is employed. [ABSTRACT FROM AUTHOR]

Background: The primary pathological alterations of Pendred syndrome are endolymphatic pH acidification and luminal enlargement of the inner ear. However, the molecular contributions of specific cell types remain poorly characterized. Therefore, we aimed to identify pH regulators in pendrin-expressing cells that may contribute to the homeostasis of endolymph pH and define the cellular pathogenic mechanisms that contribute to the dysregulation of cochlear endolymph pH in Slc26a4−/− mice. Methods: We used single-cell RNA sequencing to identify both Slc26a4-expressing cells and Kcnj10-expressing cells in wild-type (WT, Slc26a4+/+) and Slc26a4−/− mice. Bioinformatic analysis of expression data confirmed marker genes defining the different cell types of the stria vascularis. In addition, specific findings were confirmed at the protein level by immunofluorescence. Results: We found that spindle cells, which express pendrin, contain extrinsic cellular components, a factor that enables cell-to-cell communication. In addition, the gene expression profile informed the pH of the spindle cells. Compared to WT, the transcriptional profiles in Slc26a4−/− mice showed downregulation of extracellular exosome-related genes in spindle cells. Immunofluorescence studies in spindle cells of Slc26a4−/− mice validated the increased expression of the exosome-related protein, annexin A1, and the clathrin-mediated endocytosis-related protein, adaptor protein 2. Conclusion: Overall, cell isolation of stria vascularis from WT and Slc26a4−/− samples combined with cell type-specific transcriptomic analyses revealed pH-dependent alternations in spindle cells and intermediate cells, inspiring further studies into the dysfunctional role of stria vascularis cells in SLC26A4-related hearing loss. [ABSTRACT FROM AUTHOR]

Pancreatic acinar cell carcinomas are glandular and have amphophilic/eosinophilic cytoplasm, presenting acinar, solid, and trabecular structures. Unusual histological features of acinar cell carcinoma are known, such as oncocytic, pleomorphic, spindle, and clear cell variants, but their clinical significance has not been well described. A man in his 70s was referred to our hospital because of elevated serum pancreatic enzymes. Contrast‐enhanced abdominal computed tomography revealed slight swelling of the pancreatic head and suspension of the main pancreatic duct in the pancreatic body. He died only 14 days after admission. Gross findings at autopsy showed an ill‐defined tumor located in the pancreatic head, involving the gastric and duodenal walls. Peritoneal dissemination, liver metastases, and lymph node metastases were also observed. Microscopically, tumor cells had moderate‐to‐severe nuclear atypia and amphophilic cytoplasm showing pleomorphism, and diffusely proliferated in solid pattern without lumina, were admixed with spindle cells. Immunohistochemically, tumor cells including pleomorphic and spindle cells were positive for B‐cell lymphoma/leukemia 10 and trypsin. Consequently, the diagnosis was pancreatic acinar cell carcinoma with pleomorphic and spindle cells. We encountered a rare variant of pancreatic acinar cell carcinoma with pleomorphic and spindle cells. Clinically, our case showed rapid progression. [ABSTRACT FROM AUTHOR]

Leimyosarcoma (lms) is a malignant soft tissue tumor of smooth muscles. The tumor arises intramuscularly and in subcutaneous locations. It is unusual to encounter lms in head and neck region, even more infrequent to discover lms in nasal and paranasal sinuses. A case of 28 years old male with leiomyosarcoma originating from sphenoid sinus with intracranial extension is being presented with aim to highlight its rarity and to highlight the differential diagnosis and the need for prudent diagnosis in the work-up of the patient. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a mesenchymal neoplasm of fibrohistiocytic origin of moderate malignancy. The pathogenesis of DFSP involves chromosomal translocation, which leads to the formation of a fusion protein that promotes tumor growth due to increased production of platelet growth factor (PDGF). Clinically, it begins with an asymptomatic fibrous papule or firm plaques, which gradually over the course of several years become enlarged with the formation of asymmetrical multi-nodular structure of purple or red-brown color. The standards of diagnosis is a histological examination with the detection of a poor limited infiltrate indermis of a storiform architecture consisting of monomorphic spindle cells and diffuse CD34+ staining during immunohistochemistry. Complete surgical excision is considered the gold standard of treatment. Clinical case: female patient, 35 years old with a lesion in the form of a dense plaque in right subclavian area was examined by a dermatologist. The primary diagnosis was made as "morphea". In a year a slight growth of the lesion and the appearance of small firm nodules asymmetrically along the periphery were noted. According to the results of histological and immunohistochemistry studies the diagnosis of DFSP was made, the patient was referred to an oncologist for complete tumor removal.

Background: Spindle cell lipoma (SCL) is an uncommon subtype of lipomas. It usually occurs in the posterior side of the trunk such as neck, back and shoulders and rarely touches the extremities. Only four cases of SCL of the knee and no cases of SCL of the popliteal fossa have been reported in the literature. We present herein the largest case of SCL of the popliteal fossa and knee to our knowledge. Case presentation: A 75-year-old woman presented with a slow growing swelling of the left knee. Physical examination showed a 12 cm well-limited painless mass of the left popliteal fossa. The mass was pediculated with a central ulceration. MRI scan showed a tumoral mass of the left popliteal fossa with some non-dilated vessels draining to the long saphenous vein. The mass had no evidence of invasion of the underlying tissues. The patient underwent a surgical resection of the mass with postoperative recovery. The histological examination revealed a tumoral proliferation diffusely expressing CD34 without malignancy evidence, and a Ki67 proliferation index Clinical discussion: SCL is a rare histological variant of lipoma occurring mostly in the posterior side of the upper trunk. SCL of the knee or the popliteal fossa are exceptional. Clinically, it presents as a gradually increasing painless mass with a long evolving history. Histologically, these tumors are made of spindle-shaped cells, rope-like collagen fibers and mature adipocytes surrounded by a fibrous capsule. Immunohistologically, spindle cell lipomas usually show diffuse and strong expression of CD34, and to a lesser degree vimentin. The optimal treatment of SCL is a surgical marginal resection. Conclusions: SCL represents an infrequent subtype, and its atypical presentation should push clinicians to further investigations in order to rule out more worrisome malignancies.

Hearing loss is one of the top contributors to years lived with disability and is a risk factor for dementia. Molecular evidence on the cellular origins of hearing loss in humans is growing. Here, we performed a genome-wide association meta-analysis of clinically diagnosed and self-reported hearing impairment on 723,266 individuals and identified 48 significant loci, 10 of which are novel. A large proportion of associations comprised missense variants, half of which lie within known familial hearing loss loci. We used single-cell RNA-sequencing data from mouse cochlea and brain and mapped common-variant genomic results to spindle, root, and basal cells from the stria vascularis, a structure in the cochlea necessary for normal hearing. Our findings indicate the importance of the stria vascularis in the mechanism of hearing impairment, providing future paths for developing targets for therapeutic intervention in hearing loss. [ABSTRACT FROM AUTHOR]

Background: Determining reactive fibroplasia from primary neoplastic proliferation can be difficult using the cytologic features of spindle cells. Objectives: This study aimed to describe qualitative features of benign reactive spindle cells and provide a diagnostic tool to distinguish between fibroplasia and soft tissue sarcoma. Methods: Cytologic samples from cutaneous lesions with histopathologically confirmed fibroplasia were reviewed and compared with cytologic samples from confirmed low‐grade soft tissue sarcomas. The samples were obtained from the Archive of the Laboratorio Veterinario Bresciano (Brescia; Italy). Results: A total of 25 cytologic samples retrieved from the database fit the selection criteria. For comparison, 25 cytologic samples of histopathologically confirmed soft tissue sarcomas with secondary inflammation were obtained and reviewed from the same database. A low number of singularly distributed spindle cells with mild features of atypia and a high ratio of inflammatory to spindle cells was observed in confirmed cases of fibroplasia. Conclusions: Low cellularity, individualized spindle cells, and an increased inflammatory‐to‐spindle cell ratio were more indicative of fibroplasia than sarcoma. [ABSTRACT FROM AUTHOR]

Several disorders related to growth and development affect children during their early stages of life. One rare disorder that affects about 15% of children is constitutional growth delay (CGD). Its results in the affected individuals appear unusually short and young for their age. The individuals are otherwise normal and appear as their normal counterparts as age increases. Inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor that commonly affects the lungs, liver, and gastrointestinal tract. The occurrence of this tumor outside the aforementioned areas is very rare. It resembles a neoplastic tumor clinically and histologically. It is also referred to as inflammatory pseudotumor. Here, we report the case of an individual with CGD who also had an IMT involving the mandibular left canine region.

Dedifferentiated liposarcoma (DDL) with divergent dedifferentiation is uncommonly reported with variable incidences. Liposarcomas, particularly those manifesting dedifferentiation, occur in the retroperitoneum. They grow into large size, present at very late stages, and can have a poor clinical outcome. Here we present a rare case of recurrent dedifferentiated liposarcoma with dual heterologous differentiation with osteosarcoma and Chondrosarcoma in an elderly male who had initially presented with inguinal hernia. A large mass was occupying the entire abdomen, confirmed on CECT occupying the left side with loss of fat planes with the left kidney and spleen. During exploratory laparotomy, a large retroperitoneal mass occupying the whole abdomen from the left side of the retroperitoneum pushing the left and transverse colon with the mesocolon, the ureter to left side across midline was found, which was excised with the kidney preserved. Grossly multiple large fragments of tumor masses were received, ranging in size from 15 to 40 cm in their most significant dimension and weighing more than 25kgs. Extensive sampling from all the tumor masses was done for histopathology, which revealed well-differentiated liposarcoma having juxtaposed heterologous differentiation into chondrosarcomatous and osteosarcomatous areas. A final diagnosis of recurrent multifocal giant retroperitoneal dedifferentiated liposarcoma with dual heterologous differentiation with osteosarcoma and ChondrosarcomaChondrosarcoma was made. Despite recurrent multifocal tumors, the radiological information, extensive excision, detailed grossing, and histopathology helped in the diagnosis. [ABSTRACT FROM AUTHOR]

Histoid Hansen's disease is a rare form of multibacillary leprosy. It has characteristic clinical and histopathological features. It is considered to be a form of lepromatous leprosy. It can arise in lepromatous patients with inadequate or irregular treatment, who relapse after dapsone monotherapy due to dapsone resistance or may rarely occur 'de-novo'. It may present as diagnostic enigma to the physicians. We present a case of de-novo histoid leprosy in a 20-year-old female. [ABSTRACT FROM AUTHOR]

Introduction: Melanotic schwannoma (MS) is a rare neoplasm composed of Schwann cells with melanosomes in various maturation stages. While MS is typically observed in spinal nerve roots or peripheral nerves, their involvement in intraocular structures is uncommon. Here, we present a case of spontaneous globe rupture as the presenting feature of intraocular extension of a MS., Methods: Enucleation was performed to remove the lesion. Confirmatory light microscopy and immunohistochemistry were used to properly diagnose and differentiate from conventional schwannoma and uveal malignant melanoma., Results: The ocular tumor in our patient showed melanin pigmentation with positive melanocytic markers. The tumor revealed spindle and epithelioid cells proliferating in fascicles, which has previously been described for MS. No choroidal invasion, moderate nuclear pleomorphism, and a low mitotic rate were observed supporting a diagnosis of MS in our patient., Conclusion: With reports of MS with high recurrence and metastatic spread rates, there have been some suggestions that MS should be considered an aggressive malignancy. This case highlights a dramatic presentation of a rare ocular malignancy with some features of aggressiveness. Our case report endorses the importance of close monitoring for local recurrence or metastasis to prevent a poor outcome., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Spindle cell/pleomorphic lipoma (SCL/PL) is a rare histologic subtype of lipoma. It occurs in a characteristic clinical settings, arising mainly in middle-aged and older men in subcutaneous tissue of the posterior neck, upper back, and shoulders (shawl region). In women, it much more comonly develops outside the typical shawl location. The author describes a 66-year-old man with a subcutaneous tumor mass in the left back side of the neck. It consisted of bland spindle cell population situated in a fibromyxoid stroma containing thick collagen bundles. Scattered pleomorphic and multinucleated floret-like giant cells were also found. The neoplastic cells showed CD34-positive/S100-negative immunophenotype. A diagnosis of a SCL/PL was established. Subcutaneous SCL/PL is an unfamiliar tumor entity to most dermatologists. Despite the worrisome name it is a clearly benign lesion with no tendency to recur after surgical treatment. The present paper is excellent for demonstrating its unique clinical and histomorphological characteristics. [ABSTRACT FROM AUTHOR]

Infantile Myofibromatosis (IM) is a rare benign proliferative fibrous tumor of infancy arising from abnormal development of myofibroblastic and fibroblastic cell [1,2]. In this case report we present a 3-year-old boy who visited our emergency department with a history of abdominal mass started at birth as a small lump in the epigastric area massively progressed in size over the last 3 months associated with postprandial non-bilious vomiting, loss of appetite, loss of weight and decrease activity. Physical examination revealed dehydrated, cachectic child in a mild respiratory distress with decreased air entry to the right side. A well-circumscribed huge mass extending from the xiphisternum to the umbilicus was palpable in the abdomen. Laboratory workup including tumor markers were within normal range. Computed tomography scan (CT) showed evidence of a huge lobulated heterogeneous soft tissue mass straddling between the anterior mediastinum and abdominal cavity with no evidence of calcification. He was hospitalized for further assessment and complete management. In conclusion unusual presentation and diagnosis in some of pediatric tumors need an extra ordinary diagnostic approaches and surgical technique with multidisplanary team management.

Several disorders related to growth and development affect children during their early stages of life. One rare disorder that affects about 15% of children is constitutional growth delay (CGD). Its results in the affected individuals appear unusually short and young for their age. The individuals are otherwise normal and appear as their normal counterparts as age increases. Inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor that commonly affects the lungs, liver, and gastrointestinal tract. The occurrence of this tumor outside the aforementioned areas is very rare. It resembles a neoplastic tumor clinically and histologically. It is also referred to as inflammatory pseudotumor. Here, we report the case of an individual with CGD who also had an IMT involving the mandibular left canine region. [ABSTRACT FROM AUTHOR]

Synovial sarcoma, an uncommon malignant tumor, can occur in various anatomical sites but mostly involves joints. It is invasive locally and tends to spread to other sites. We report a male patient, 56 years old, who had swelling above his right ankle. Soft tissue edema was observed across the lateral malleolus in imaging examinations. A synovial sarcoma was diagnosed on histopathology. A complete resection was performed and postoperative treatment with doxorubicin and cyclophosphamide was given for 2 months. The 5-year survival rate is only expected between 27-55%. Furthermore, many authors concur that synovial sarcomas are among the most frequently misdiagnosed soft tissue cancers due to their sluggish growth pattern, benign radiographic appearance, capacity to fluctuate in size, and potential to induce pain similar to that brought on by ordinary trauma. Therefore, the case emphasizes the need for additional study to identify the best diagnostic approaches for synovial sarcoma., Competing Interests: The authors declare no competing interests., (Copyright: Shivali Kalode et al.)

Disappointment in interpersonal relationships may arise from a gap between the intentions and actions or perceptions that each person has of their own intentions and actions and those of others (Intention and Behaviour Gap). This gap can originate from: (a) a diminished or lack of insight or awareness of own emotions and behaviours, and it’s effect on others, (b) lack of awareness of the intention or the desired outcomes and (c) the gap existing between the two people as well as the (d) inability to change behaviour. This gap could be related to behaviours people have that have become known and culturally accepted as rigid and inflexible. Consequently, the person focuses on their intention with little to no self-knowledge of the displayed behaviour, thus judging their own actions on their good personal intention while judging others based on their perceptions rather than the others’ intentions. This inclination amounts to self-righteousness and can increase the likelihood of frustration and anger towards the perceived bias and lack of fairness towards others. In turn, this might cause interpersonal discord, resentment, misunderstanding, and conflict. To address this behavioural gap, this study proposes the application of a 3-Dimensional, Visualisation and Simulation Model (3DVSM) to enhance insight into own and others behaviours and actions, followed by connecting one’s actions to the desired intention. The ultimate goal is to instil a desire for modifying one’s behaviour, thus reducing interpersonal conflict by shortening the gap between intention and action.

Abstract Background High-grade spindle cell sarcomas are a subtype of rare, undifferentiated pleomorphic sarcomas (UPSs) for which diagnosis is difficult and no specific treatment strategies have been established. The limited published data on UPSs suggest an aggressive clinical course, high rates of local recurrence and distant metastasis, and poor prognosis. Case presentation Here we present the unusual case of a 45-year-old male patient with a lumbosacral UPS extending into the sacrum. An initial diagnosis of a low-grade malignant spindle cell tumor was based on a tumor core biopsy. After complete extensive resection, the diagnosis of an UPS of the lumbosacral region was confirmed by excluding other types of cancers. Despite treatment with neoadjuvant radiotherapy, extensive resection, and adjuvant chemotherapy, the patient presented with multiple pulmonary metastases 3 months after surgery. The patient then began treatment with crizotinib at an oral dose of 450 mg per day, based on the detection of a LMNA-NTRK1 fusion gene in the tumor by next-generation sequencing. Over 18 months of follow-up through July 2018, the patient maintained a near-complete clinical response to crizotinib. Conclusions The LMNA-NTRK1 fusion was likely the molecular driver of tumorigenesis and metastasis in this patient, and the observed effectiveness of crizotinib treatment provides clinical validation of this molecular target. Molecular and cytogenetic evaluations are critical to accurate prognosis and treatment planning in cases of UPS, especially when treatment options are limited or otherwise exhausted. Molecularly targeted therapy of these rare but aggressive lesions represents a novel treatment option that may lead to fewer toxic side effects and better clinical outcomes.

Objective: Atypical polypoid adenomyomas (APAs) are uncommon tumours consisting of atypical endometrioid glands and fibromyomatous stroma. Identifying the biphasic nature of atypical glandular components and spindle mesenchymal components without atypia is crucial for the cytological diagnosis of APA. We investigated the utility of lesion‐targeted cytology (LTC) to directly collect firm spindle components. Methods: We recruited seven consecutive surgical patients who underwent cytological examinations before surgery and were diagnosed with APA on postoperative histological examinations. Cytological smears were obtained by routine sampling in five cases and by targeted sampling using transvaginal ultrasonography, that is, LTC, in two cases. We retrospectively analysed the cytological findings from our cases and compared them to those of APA cases previously reported in the English literature. Results: Among 5/7 cases that involved routine cytological sampling, normal cytological findings were found in 2 and atypical glandular cells were found in 3, but spindle cells from mesenchymal components were not detected. In contrast, among 2/7 cases in which sampling involved LTC, spindle cells without atypia, in addition to atypical glandular cells were found. Conclusions: Lesion‐targeted cytology is useful to assess mesenchymal components of APAs and may improve the cytological diagnosis of APA. Accurate cytological diagnosis of atypical polypoid adenomyomas (APAs) requires identifying the biphasic nature with atypical glandular components and spindle mesenchymal components. However, mesenchymal components are more difficult to collect than glandular cells. Lesion‐targeted cytology is useful to assess mesenchymal components of APAs and may improve the cytological diagnosis of APA. [ABSTRACT FROM AUTHOR]

• Gangliocytic paraganglioma is an exceedingly rare tumors that is mainly arises from the second part of the duodenum. • The tumor was first described by Dahl et al. in 1957. • The tumor has a characteristic 3 distinct types of cells: spindle cells, epithelial cells, and ganglion cells. • The tumor must be differentiated from other tumors. • Although it is a benign tumor but some cases have lymphatic or distant organ metastases. Gangliocytic paraganglioma is a rare tumors mainly arises from the second part of the duodenum. The tumor is generally benign, but some cases are associated with lymph node or distant metastasis. It must be differentiated from other tumors. The main treatment is good local surgical resection. Most patients have good prognosis after an appropriate resection. A 47-year-old male patient presented with recurrent attacks of epigastric pain and melena. The general examination revealed pallor with no jaundice. The abdomen was soft with no tenderness. There were no stigmata of chronic liver diseases. The hemoglobin level was 8 gm/dl with normal liver and renal functions. The patients received compatible blood. Endoscopy showed a large pedunculated polyp with ulceration in the second part of the duodenum just distal to ampulla of Vater. During surgery, the anterior wall of duodenum was opened, and a 4 cm pedunculated polyp was excised which was arising from the posterior wall of the duodenum just distal to the ampulla of Vater. The frozen section examination revealed a benign lesion. The final histopathology result was consistent with the diagnosis of gangliocytic paraganglioma. The patient had smooth post-operative recovery and no postoperative complications. Gangliocytic paraganglioma is a rare tumor which mostly affects the second part of the duodenum. The tumor is considered benign but the possibility of the malignancy with lymphatic and distant metastases must be excluded before any surgery. The management plan must be addressed carefully and continuous follow up is recommended. [ABSTRACT FROM AUTHOR]

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The diagnosis of amelanotic blue nevi can be challenging based solely on clinical observation as these lesions are often confused with dermatofibromas, neurofibromas, or other neoplasms.2 Histologically, amelanotic blue nevi share features with other skin pathology, including amelanotic and/or spindle cell melanoma, Spitz nevus, neurofibroma, and dermal scar.1 Successful diagnosis requires clinical awareness of such variants and appropriate application of histologic discriminants. Keywords: amelanotic blue nevus; immunohistochemistry; MART-1; SOX10; spindle cells EN amelanotic blue nevus immunohistochemistry MART-1 SOX10 spindle cells 1312 1314 3 11/11/21 20210901 NES 210901 CASE PRESENTATION An otherwise healthy 3 year-old girl presented to clinic with an asymptomatic papule over the sacral area on the back. Histologically, these nevi demonstrate spindle cell proliferation, and five predominant histological patterns have been identified, which resemble dermatofibroma, sclerotic fibroma, common blue nevus, scar, and cellular blue nevus.1 The common blue nevus-like pattern, as seen in this case, contains spindle cells with intercalating collagen bundles. [Extracted from the article]

Giant cell-rich osteosarcoma (GCRO) is an exceedingly rare histological variant of conventional primary osteosarcoma. It constitutes about 1%–3% of all osteosarcomas, and is extremely uncommon in the maxillofacial region. The unusual histopathological appearance and the rarity of the lesion poses a great diagnostic challenge. This article aims to present a rare case of GCRO involving the mandible in a 52-year-old male patient. [ABSTRACT FROM AUTHOR]

Spindle cell neoplasms of the head and neck region are relatively rare, yet there importance cannot be doubted. Spindle cells are of mesenchymal origin and constitute a part of the body’s connective tissue. Soft tissue spindle cell neoplasms may range from reactive lesions to benign and malignant tumours. This heterogenous group of lesions include those of neural, fibroblastic, myofibroblastic, myogenic and epithelial tumours. As the spindle cells contain both benign and malignant mimics, misclassification has the potential to result in either under or over treatment of the patient. The aim of this review is to evaluate available data on spindle cell neoplasms, to gain more insight into the molecular pathogenesis and immunohistochemistry of these lesions, and to integrate recent knowledge on spindle cell neoplasms and their emerging concepts for future prospects. An effort has also been made to add a few relevant neoplasms to the present working classification.

Malignant spindle cell tumours of the larynx are rare, of which the most common are Spindle Cell Squamous Carcinomas (SCSCs). Sarcomas are extremely infrequent in the larynx. Composite tumours, further, are quite unheard of, except for rare case reports. This case report describes a spindle cell malignancy of the larynx in an elderly male, which posed a diagnostic difficulty due to its unique morphological and immunohistochemical features. The tumour was superficial, polypoidal with the proliferation of malignant spindle cells and abundant mitoses under an ulcerated epithelium. Occasional squamous cell rests with keratin pearls were present. Spindle cells were strongly immunoreactive for vimentin, smooth muscle actin and squamoid cells were p63 positive. Desmin was negative. The dilemma in diagnosis was of a SCSC with smooth muscle differentiation against a composite tumour. The tumour also turned out to be positive for Epstein Barr Virus Latent Membrane Protein-1 (EBV-LMP1), establishing a noteworthy association.

High-grade spindle cell sarcomas are rare undifferentiated pleomorphic cancers that present a treatment challenge to urological practices, especially when they present in the pelvis. We report a 46-year-old male patient who presented to our urology clinic with urinary retention after having a Foley catheter placed at an outlying facility. A voiding trial was attempted, but the patient failed this trial. This failure resulted in cystoscopy with bilateral retrograde pyelograms, which revealed a compressed bladder due to extrinsic compression. This finding had been evaluated with a computed tomography (CT) scan with and without intravenous contrast that showed a 14 cm pelvic mass with bladder displacement and compression. A fine needle aspiration was done at this outlying facility, prior to referral to our office, and it confirmed spindle cell pathology. The mass was surgically excised with the histology revealing a pelvic spindle cell sarcoma with positive surgical margins. Further, metastatic work-up with CT/positron emission tomography revealed bone and lung metastasis. The patient is currently undergoing chemotherapy and radiation. In this case study, we will review staging, management, differential diagnosis, chemotherapy, and radiation.