Extraskeletal myxoid chondrosarcoma metastasis to a Meckel's diverticulum adenocarcinoma / Metástasis de condrosarcoma mixoide extraesquelético a un adenocarcinoma de divertículo de Meckel

Extraskeletal myxoid chondrosarcoma is a rare soft tissue tumour with a high local and distant metastasis rate and limited response to chemotherapy. Meckel's diverticulum is the most frequent congenital anomaly, and it is associated with a considerable risk of malignant transformation. In this case report, we describe a 50-year-old female patient with a history of extraskeletal myxoid chondrosarcoma of the lower limb and metastasis to the forearm who went to the emergency department with abdominal pain. The investigations revealed a caecal volvulus. A lesion in the middle third of the ileum was incidentally discovered and removed during surgery. Pathology examination revealed a Meckel's diverticulum adenocarcinoma, with metastasis of extraskeletal myxoid chondrosarcoma. Resection was complete; however, the patient had diffuse metastatic pulmonary disease and died eight months later due to disease progression. This mechanism of tumour-to-tumour metastasis is described in other locations, but, regarding the Meckel's diverticulum, this is a unique situation, previously unreported in the literature. (AU)

El condrosarcoma mixoide extraesquelético es un tumor de tejidos blandos poco frecuente, con una elevada tasa de recurrencia y metástasis a distancia y una respuesta limitada a la quimioterapia. El divertículo de Meckel es la anomalía congénita más frecuente y se asocia a un riesgo considerable de transformación maligna. En este caso clínico describimos a una paciente de 50 años con antecedentes de condrosarcoma mixoide extraesquelético de miembro inferior y metástasis en el antebrazo que acudió al servicio de urgencias por dolor abdominal. La exploración reveló un vólvulo cecal. Se descubrió incidentalmente una lesión en el tercio medio del íleon, que se extirpó durante la intervención quirúrgica. El examen patológico reveló un adenocarcinoma de divertículo de Meckel, afectado por metástasis de condrosarcoma mixoide extraesquelético. La resección fue completa; sin embargo, la paciente presentaba enfermedad pulmonar metastásica difusa y falleció ocho meses después debido a la progresión de la enfermedad. Este mecanismo de metástasis entre tumores está descrito en otras localizaciones, pero en lo que respecta al divertículo de Meckel, se trata de una situación única en la literatura. (AU)

Cardiac Morbidity and Mortality in Patients with Sarcoma: A Population-Based Study.

Background: Soft tissue sarcoma (STS) is a rare malignancy that affects soft tissues. It encompasses various subtypes and requires different treatment strategies. Doxorubicin is a commonly used anthracycline in the management of localized and metastatic STS. However, high doses of doxorubicin are associated with cardiotoxicity, which can significantly impact patients' long-term outcomes. This study aimed to evaluate doxorubicin's effect on cardiac function in patients with sarcoma and to correlate the frequency of cardiotoxicity with potential risk factors. Methods: A retrospective analysis was conducted on patients with sarcoma who were treated with doxorubicin between 2016 and 2022 at King Abdulaziz Medical City in Saudi Arabia. Patient demographic information, comorbidities, cardiac measurements, laboratory values, systemic therapy, and treatment outcomes were collected from electronic medical records. A statistical analysis was performed to assess the association between cardiotoxicity and various factors. Results: A total of 133 patients were included in the study, with a median age of 30 years. Cardiotoxicity was observed in 9% of the patients. Female patients had a significantly higher risk of developing cardiotoxicity. Patients with a higher Eastern Cooperative Oncology Group (ECOG) performance status and lower troponin I levels also had an increased risk of cardiotoxicity. However, there was no significant association between cardiotoxicity and the number of chemotherapy cycles, total cumulative dose of doxorubicin, or history of radiation. Furthermore, patients with cardiotoxicity had a higher risk of mortality. The overall survival of the patients was 18 months. Conclusion: Doxorubicin-associated cardiotoxicity is a concern for patients with sarcoma. Female patients and patients with a higher ECOG performance status are at an increased risk of developing cardiotoxicity. Careful monitoring and risk assessment are crucial for mitigating the adverse effects of doxorubicin treatment in patients with sarcoma. Future studies are warranted to validate these findings and explore preventive strategies for doxorubicin-induced cardiotoxicity in patients with sarcoma.

Ewing's sarcoma of the parotid gland: A rare entity with review of the literature.

Background: Ewing's sarcoma is a rare malignant entity. Extraosseous Ewing's sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing's sarcoma of the parotid gland is even rarer. To the best of our knowledge, we reported the first case of extraskeletal ES originating from the parotid gland in the Tunisian literature. Case report: We report a rare case of EES of the parotid gland in a 35-year-old female. She presented with left parotid tumefaction. Physical examination revealed solid and fixed mass associated with facial paralysis. Magnetic resonance imaging illustrated a left intra-parotid process occupying the entire gland measuring 42 mm infiltrating the masseter and pterygoid muscles. The patient had a total left parotidectomy with ipsilateral triangular lymph node dissection. The definitive pathological examination and the immunohistochemical staining confirmed a primary peripheral neuroectodermal tumor or PNET with the presence of a specific EWING/PNET-type translocation in 60% of the tumor cells. She had an adjuvant chemotherapy (four cycles of vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide) followed by external radiotherapy. Conclusion: A clinical and radiological follow-up by cervical MRI was done every 3 months and The 10-month follow-up showed no locoregional and distant recurrence.

Introduction: Le sarcome d'Ewing est une entité maligne rare. La localisation extra osseuse en particulier la région de la tête et du cou est caractérisée par son agressivité locorégionale. Nous rapportons le cas d'un sarcome d'Ewing de la parotide. A notre connaissance, il s'agit du premier cas rapporté dans la littérature tunisienne. Presentation du cas: Il s'agit d'une femme âgée de 35 ans qui a consulté initialement pour une tuméfaction au niveau de la glande parotide gauche. L'examen clinique a révélé une masse sous angulomandibulaire associée à une paralysie faciale périphérique gauche. Une IRM parotidienne a objectivé un processus intra-parotidien gauche occupant l'entièreté de la glande mesurant 42 mm mal limitée infiltrant modérément le muscle masséter et ptérygoïdiens. Le bilan d'extension était sans anomalie. La patiente a eu une parotidectomie gauche large avec un curage triangulaire homolatéral. L'examen anatomopathologique définitif et l'étude par hybridation in situ en fluorescence (FISH) ont confirmé la présence d'une translocation spécifique type EWING/PNET dans 60% des cellules tumorales. La patiente a été traitée par une chimiothérapie type VDC/IE (vincristine, doxorubicine, cyclophosphamide en alternance avec ifosfamide, et etoposide) suivie d'une radiothérapie externe. Un suivi clinique et radiologique trimestrielle n'a montré aucune récidive locorégionale ni à distance à ce jour soit à 10 mois de recul. Conclusion: Une surveillance clinique et radiologique trimestrielle a été faite et le contrôle à 10 mois n'a pas montré de récidive locorégionale ou à distance.

A Rare Coexistence of Hybrid Tumor Low-grade Fibromyxoid Sarcoma/Sclerosing Epithelioid Fibrosarcoma and Hibernoma in the Same Thigh: A Case Report.

Introduction: We encountered a unique case of a patient with two distinct tumors coexisting in the same thigh. To the best of our knowledge, this combination of tumors in the same anatomical region has not been previously described in the literature. Case Report: This case report describes a 38-year-old Caucasian male with a painless mass in his right thigh, which was later diagnosed as a hybrid tumor composed of low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma, as well as a second tumor, which was diagnosed as a hibernoma. The patient underwent neoadjuvant chemotherapy and surgical excision, followed by adjuvant radiotherapy and treatment for metastatic recurrence. Conclusion: The rarity of this case highlights the need for interdisciplinary collaboration and further investigations into the behavior and management of hybrid tumors. This case also underscores the importance of an accurate histological diagnosis aided by immunohistochemistry and molecular analyses.

Philosophies And Surgical Techniques for Ewing's Sarcoma of Spine with Review of Literature.

Introduction: Ewing sarcoma (ES) is a malignant and aggressive bony tumor affecting the most common age group of 5-20 years. It constitutes 10%-15% of all bone sarcomas and is the second most common primary malignant bone tumor after osteosarcoma. It usually presents with pain, which is typically constant and progressive in nature. The primary source of pain is due to the instability of the spine to support the weight of the body, the vertebral body's expanding cortices due to the growing mass, compression of nerve roots due to tumour mass, pathologic fractures, spinal cord compression, and invasion of tissue by the tumour mass. Methods: We reviewed the literature on Ewing's Sarcoma of the spine to evaluate its etiology, clinical presentations, differential diagnosis, imaging modalities and management with chemotherapy, radiotherapy, and surgical management. PubMed, EMBASE, Google Scholar and Cochrane key articles were searched. Keywords like 'Ewing's Sarcoma,' 'Spine,' 'etiology,' 'treatment,' 'surgical management,' and 'en bloc resection' were used. Discussion: The current management of Ewing's sarcoma of the spine usually involves three primary modalities: combination chemotherapy, surgery and/or radiotherapy. Recent improvements in combination chemotherapy (vincristine, doxorubicin, cyclophosphamide +/- Ifosfamide and etoposide) are among the most significant factors for improving survival. Also, recent advancements in radiotherapy, instrumentation, and fusion techniques in surgical management have been demonstrated to improve local disease control and overall survival. Conclusion: Primary Ewing sarcoma of the spine is a rare condition affecting the most common age group of 5-20 years, accounting for 1-3 cases/million/year. About 5 % of cases have spine involvement. Recent improvements in combination chemotherapy have improved the overall survival rates. Enbloc resection and/or radiotherapy have improved local control of the disease.

Amputation surgery associated with shortened survival in patients with localized extremity bone sarcoma.

Background: This study assesses survival rates among patients with localized extremity bone sarcoma who have undergone amputation, pinpointing subpopulations that are disproportionately affected by amputation-related survival disparities. Methods: Examination of data was conducted using the Surveillance, Epidemiology, and End Results (SEER) program, analyzing records of 3765 patients diagnosed with localized extremity bone sarcoma between 2000 and 2019. Of these, 857 received amputations (Amputation cohort), and 2908 underwent limb-sparing surgeries. Propensity score matching, considering demographic and clinical features, was utilized to ensure a fair comparison. Results: Following propensity score matching, the study focused on 1714 cases. The Amputation cohort was observed to have significantly poorer survival rates (Cancer-Specific Survival [CSS]: Hazard Ratio [HR] = 1.28, 95% Confidence Interval [CI]: 1.05-1.55; Overall Survival [OS]: HR = 1.37, 95% CI: 1.15-1.63). Subsequent subgroup analysis indicated that individuals with tumors exceeding 8 cm in size or those located in the lower limbs were notably at a higher risk of shortened survival (for tumors >8 cm - CSS: HR = 1.32, 95% CI: 1.02-1.71; OS: HR = 1.39, 95% CI: 1.09-1.76; for lower limb tumors - CSS: HR = 1.25, 95% CI: 1.01-1.54; OS: HR = 1.33, 95% CI: 1.11-1.61). Conclusions: Our findings demonstrate that patients diagnosed with localized extremity bone sarcoma undergoing amputation exhibit lower survival rates, especially in cases involving tumors of greater size or those situated in the lower limbs. In patient groups where amputation is inevitable, careful follow-up is required after surgical intervention.

Embryonal Rhabdomyosarcoma of the Prostate: Clinico-Pathological Highlights with Review of Literature.

Rhabdomyosarcoma (RMS) is the third most common extra-cranial sarcoma occurring in childhood, adolescents, and young adults (AYAs); and is rare in adults. Literature about RMS mainly considers RMS in AYAs, either with that in the children or adults, even though histological, molecular, and clinical characteristics of RMS in AYAs are significantly different from either of the two. Herein, we report a case of prostatic embryonal RMS, in a 17-year-old boy, along with the review of literature of prostatic RMS, with emphasis on AYAs. Our patient presented with clinical complaints of acute urinary retention, Grade IV prostatomegaly and, low serum prostate-specific-antigen (0.11ng/dl). The diagnosis was clinched by prostatic biopsy, which revealed diffuse 'small round blue cell' tumour admixed with larger rhabdomyoblasts, displaying positivity for desmin and myogenin, on immunohistochemistry. Clinicians should be mindful that RMS is found in all age groups ranging from childhood to adults; however, the clinical, histological, and molecular features are different. RMS in AYAs is often treated according to the guidelines provided for the paediatric age group. Treatment mostly comprises a multimodality approach, including surgery with/without chemo- and radiotherapy. Prognosis in AYAs is worse than in children but is better than in adults. Thus, early diagnosis gains utmost importance to provide comparatively more probability of rendering treatment and, hopefully, a better quality of life.

Vaginal Endometrial Stroma Sarcoma: A Case Report of A Rare Disease.

Vaginal endometrial stromal sarcoma (VESS) arising in the vagina is an extremely rare extrauterine endometrial stromal sarcoma, Stroma Sarcoma. To the best of our knowledge, there have been only a few reported cases in the literature. Therefore, we herein report a case of VESS originating in the vagina. A 27-year-old woman complained of heavy and intermittent vaginal bleeding and abdominal swelling all of six months duration. A mass that was firm to hard in consistency was found in the middle and upper segments of the anterior, right lateral, and posterior fornices and wall of the vagina. An excisional biopsy was taken and the immunohistochemistry result revealed VESS. A cystoscopy was done which revealed polypoid metastatic nodules in the bladder (this put the stage of the disease at IV). She also had laparotomy following features of mechanical obstruction from previous pelvic and intraperitoneal adhesions which were formed from previous laparotomy.

Natural history of undifferentiated pleomorphic sarcoma: Experience from the US Sarcoma Collaborative.

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) is a relatively rare but aggressive neoplasm. We sought to utilize a multi-institutional US cohort of sarcoma patients to examine predictors of survival and recurrence patterns after resection of UPS. METHODS: From 2000 to 2016, patients with primary UPS undergoing curative-intent surgical resection at seven academic institutions were retrospectively reviewed. Epidemiologic and clinicopathologic factors were reviewed by site of origin. Overall survival (OS), recurrence-free survival (RFS), time-to-locoregional (TTLR), time-to-distant recurrence (TTDR), and patterns of recurrence were analyzed. RESULTS: Of the 534 UPS patients identified, 53% were female, with a median age of 60 and median tumor size of 8.5 cm. The median OS, RFS, TTLR, and TTDR for the entire cohort were 109, 49, 86, and 46 months, respectively. There were no differences in these survival outcomes between extremity and truncal UPS. Compared with truncal, extremity UPS were more commonly amenable to R0 resection (87% vs. 75%, p = 0.017) and less commonly associated with lymph node metastasis (1% vs. 6%, p = 0.031). R0 resection and radiation treatment, but not site of origin (extremity vs. trunk) were independent predictors of OS and RFS. TTLR recurrence was shorter for UPS resected with a positive margin and for tumors not treated with radiation. CONCLUSION: For patients with resected extremity and truncal UPS, tumor size >5 cm and positive resection margin are associated with worse survival OS and RFS, irrespectively the site of origin. R0 surgical resection and radiation treatment may help improve these survival outcomes.

RAD51C and MYST3 Mutations in a Case of Desmoid-Type Fibromatosis With No Mutation in CTNNB1 or APC.

Most cases of desmoid-type fibromatosis (DTF) exhibit a mutation in APC or CTNNB1. We report a case of mesenteric DTF in which no mutation in APC or CTNNB1 was found, but a germline variant of uncertain significance (VUS) in RAD51C and a subclonal mutation in MYST3 were identified. Whether these genetic changes are important in DTF in this case, or whether genetically conventional DTF cells were present at a density below detection is unknown; it will be of interest to see results in further studies of wild-type APC/CTNNB1 cases.

Secondary hematological malignancies in patients with sarcoma: A single­center retrospective study.

The present retrospective study investigated the clinical features and prognosis of secondary hematological malignancies (SHMs) in patients with sarcoma at Korea Cancer Center Hospital (Seoul, South Korea). Patients who had been diagnosed with SHMs after having received treatment for sarcoma between January 2000 and May 2023 were enrolled. Clinical data were collected from the patients' medical records. Clinical characteristics were analyzed, including SHM incidence, type and prognosis. Of 2,953 patients with sarcoma, 18 (0.6%) were diagnosed with SHMs. Their median age at the time of sarcoma diagnosis was 39.5 (range, 9-72) years, and 74% (n=14) of these patients were male. The histological features of sarcoma varied, with osteosarcoma diagnosed in nine patients (50%). All patients with sarcoma underwent surgical treatment, and 16 (88.8%) received chemotherapy. The most common type of SHMs was acute myeloid leukemia (n=6; 33.3%), followed by myelodysplastic syndrome (n=5; 27.7%). The median latency period between the sarcoma diagnosis and SHM identification was 30 (range, 11-121) months. A total of 13 (72.2%) patients received treatment for the SHM. The median overall survival after SHM diagnosis was 15.7 (range, 0.4-154.9) months. The incidence of SHMs in sarcoma in the present study was consistent with that reported previously. The presence of SHMs was associated with a poor patient prognosis, especially if treatment for SHMs was not administered.

Primary intramedullary extradural Ewing sarcoma.

Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical findings, and treatment of a primary intradural extramedullary Ewing sarcoma with a unique intracranial metastatic component in a pediatric patient. A 14-year-old girl with a history of mood disorders presented to the emergency department with a 3-week history of neck torticollis, cervical pain, paresis, and paresthesia of the upper and lower extremities on the left side. Initially, non-organic causes such as somatization or conversion disorder were suspected. She returned 3 months later when her symptoms worsened. MRI of the head and spine was performed, and demonstrated the presence of a suprasellar, retro-chiasmatic mass lesion. There was also diffuse leptomeningeal enhancement, another well-defined intradural extramedullary lesion the sacral region and several multifocal cauda equina soft tissue nodules. The patient first underwent surgery. The patient was also treated with a combination of chemotherapy (vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE)) and radiation as per the Children's Oncology Group AEWS1221 protocol. Most recent imaging conducted 22 months after the initial mass discovery revealed improvement of the suprasellar mass lesion with residual stable appearance of the prominence and enhancement of the pituitary stalk and tuber cinereum. There was interval improvement of the spinal lesions with no convincing residual. Clinically, at almost three years since initial imaging findings, and 25 months since completing treatment, she is stable from an oncology perspective.

Utility of Indocyanine Green for Sentinel Lymph Node Biopsy in Pediatric Sarcoma and Melanoma.

BACKGROUND: Indocyanine green (ICG) is a fluorescent dye with increasing use for adult sentinel lymph node biopsy (SLNB). The utility of ICG in pediatric oncology remains understudied. We aim to describe our experience using ICG for SLNB in pediatrics versus standard blue dye. METHODS: A retrospective review of pediatric patients with melanoma or sarcoma who underwent SLNB with technetium plus ICG or blue dye from 2014 to 2023 at a large academic children's hospital was conducted. RESULTS: Twenty-four patients were included; 58.3% were male with median age 13 years (range 4-21 years). The majority had a melanocytic tumor (91.7%) and 8.3% had sarcoma. All patients received technetium with concomitant blue dye (62.5%) or ICG (37.5%). ICG more reliably identified radioactive SLNs, compared to blue dye (mean 100% vs 78.3 ± 8.3%, p = 0.03). There was no significant difference in median operative time (ICG 82 min [68-203] vs blue dye 93 min [78-105], p = 0.84). Seven patients had positive SLNs (29.2%), with recurrence in 2 patients (8.3%) and 1 death (4.2%). There were no adverse events. CONCLUSION: ICG-directed SLNB in children is a safe and effective alternative to blue dye. Use of ICG did not add to operative time, and more often identified sentinel nodes versus blue dye. TYPE OF STUDY: Original Research Article, Retrospective Comparative Study. LEVEL OF EVIDENCE: III.

Advances in Systemic Therapy in Pancreatic Cancer.

Substantial progress has been made toward understanding biology and developing new therapies for pancreatic ductal adenocarcinoma (PDAC). In this review, new insights from genomic profiling, as well as implications for treatment and prognosis, are discussed. New standards of care approaches with a focus on drug therapies are discussed for the treatment of resectable and advanced PDAC. The role of targeted and immune therapies remains limited; cohorts likely to benefit from these approaches are discussed. Promising, preliminary results regarding experimental therapies are reviewed.

18 F-Fluorodeoxyglucose Uptake in Bilateral Diaphragmatic Crura: A Relatively Uncommon Benign Variant Noted in a Treated Case of Extraosseous Paraspinal Ewing's Sarcoma.

A toddler was diagnosed with extraosseous Ewing's sarcoma, primary large epidural paraspinal soft tissue in the lumbar region encasing the cord and neural foramen from D12-L1 to L4-L5. After eight cycles of induction chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with etoposide and ifosfamide, 18 F-FDG positron emission tomography/computed tomography ( 18 F-FDG-PET/CT) scan confirmed no active disease. Later external beam radiotherapy (EBRT) at D10-L5 was completed. At 3 months follow-up, 18 F-FDG-PET/CT reconfirmed no residual/active disease; however, a new incidental finding of diffuse benign bilateral diaphragmatic 18 F-FDG uptake was noted in the clinically asymptomatic patient, which remained unexplained.

Intravascular Leiomyoma Considered Preoperatively as Uterine Sarcoma: A Rare Case.

Intravascular leiomyoma (IVL) is usually defined as a histologically benign leiomyoma that originates in a uterine fibroid or the intrauterine vein wall and grows and expands intravenously. We report a case in which pelvic IVL was detected early and discuss the early diagnosis of and best treatment for this tumor.

Elderberry interaction with pazopanib in a patient with soft­tissue sarcoma: A case report and literature review.

Elderberry flower extract is marketed as an herbal supplement with purported benefits in boosting the immune system. The use of elderberry increased during the coronavirus pandemic. However, the interaction of elderberry with cytotoxic medicines has remained elusive. Pazopanib is a multikinase inhibitor approved for patients diagnosed with soft-tissue sarcoma. The present study reported on the case of a middle-aged woman diagnosed with localized intermediate-grade sarcoma of the left sartorius muscle who received neoadjuvant pazopanib with radiation therapy. The patient had no other medical comorbidities and only took over-the-counter (OTC) elderberry supplements for numerous years to 'boost' her immune system. She started pazopanib at 400 mg per os (PO) daily, which was increased to 800 mg PO daily after a week. By week three on pazopanib, the patient reported intense nausea and a number of loose stools, requiring anti-nausea medication. By the fourth week on pazopanib, laboratory tests showed grade 3 liver injury, as demonstrated by a fivefold rise in liver enzymes along with severe nausea and loose stools. All medications, including elderberry supplement, were stopped. Within two weeks of stopping all medicines, the liver enzymes started normalizing within two weeks and were normal by the end of four weeks. Pazopanib treatment was resumed without the recurrence of side effect. Pazopanib is metabolized in the liver via the cytochrome P 450 (CYP)3A4 enzyme pathway. Hence, potent inhibitors of CYP3A4 are avoided for concurrent use with pazopanib. Small in vitro studies on elderberry extracts have shown weak inhibition of CYP3A4. However, considering the wide usage of elderberry and the availability of mixed supplements OTC, it is essential to pursue clinical studies in cancer patients to understand the interactions of elderberry extracts with cytotoxic medicines. In this report, the scientific evidence behind the use of elderberry was reviewed and a hypothesis of its interaction with pazopanib was proposed.

[Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion]. / Pervichnaya legochnaya miksoidnaya sarkoma so sliyaniem genov EWSR1-CREB1.

Primary pulmonary myxoid sarcoma with EWSR1-CREB1 fusion is an extremely rare tumor. Its clinical manifestation is unspecific and only molecular genetic method can proof this diagnosis. This paper describes an unusual clinical presentation of primary pulmonary myxoid sarcoma in a 68-year-old patient with involvement of both lungs.

Cytomorphology of metastatic colonic MXD4::NUTM1-rearranged sarcoma.

This report describes the cytologic features of a recently described MXD4::NUTM1-rearranged colonic sarcoma metastatic to the midclavicular soft tissue. Thirteen years ago, a 65-year-old woman presented with a cecal mass and subsequent liver mass. The cecal mass was diagnosed as malignant undifferentiated spindled and epithelioid neoplasm based on morphology and lack of tumor immunoreactivity with a panel of epithelial, smooth muscle, skeletal, melanoma, hematologic, and GIST markers. The liver mass showed morphologic and immunophenotypic similarity to the epithelioid component of the patient's cecal mass, albeit devoid of the spindled component. Fine needle aspiration of the midclavicular soft tissue mass showed singly scattered to clustered epithelioid to rhabdoid tumor cells with centrally to eccentrically located nuclei, prominent nucleoli, and moderate eosinophilic cytoplasm. Immunohistochemical stains performed on the concurrent biopsy showed the tumor cells were positive for NUT and negative for all other additional markers with retained normal expression of SMARCA2 and SMARCA4. Next-generation sequencing showed the presence of MXD4::NUTM1 gene fusion. Due to the identical cytomorphologic findings with the epithelioid component of the patient's prior cecal and liver masses, the tumor was deemed as consistent with a NUTM1-rearranged sarcoma. To our knowledge, this case represents the first reported cytologic features of a NUTM1-rearranged sarcoma on fine needle aspiration. Familiarity with the cytologic features, inclusion of this entity in the differential diagnosis of tumors with epithelioid and/or rhabdoid morphology, and performance of ancillary tests (immunohistochemistry and molecular) will be helpful in arriving at the right diagnosis.