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1.
Can Vet J ; 65(7): 661-666, 2024 Jul.
Article de Anglais | MEDLINE | ID: mdl-38952763

RÉSUMÉ

Carcinosarcomas are very rare tumors in dogs. Although carcinosarcomas with melanocytic differentiation arising from organs other than the thymus have been described in humans, this type of tumor has not been reported in dogs in any part of the body. We observed such a tumor in the cranial mediastinum of an 11-year-old spayed female dachshund. The dog was admitted to the clinic because of coughing, sporadic regurgitation, and dyspnea. Thoracic ultrasonography and computed tomography revealed a large mediastinal mass that was surgically removed via sternotomy. The tumor was of thymic origin and demonstrated 3 distinct components: an epithelial component positive for pancytokeratin (AE1/AE3) and high molecular weight cytokeratin (CK5/CK6) with some cystic spaces; a mesenchymal component positive for vimentin; and in association with the epithelial part, a minor melanocytic component positive for Melan A. Histologic metastasis of the epithelial and melanocytic components was present within a tracheobronchial lymph node. The dog died 105 d after surgery, after an episode of acute dyspnea. Key clinical message: To the authors' knowledge, this is the first report of thymic carcinosarcoma with melanocytic differentiation.


Carcinosarcome thymique avec différenciation mélanocytaire chez un chienLes carcinosarcomes sont des tumeurs très rares chez le chien. Bien que des carcinosarcomes avec différenciation mélanocytaire provenant d'organes autres que le thymus aient été décrits chez l'homme, ce type de tumeur n'a été rapporté chez le chien dans aucune partie du corps. Nous avons observé une telle tumeur dans le médiastin cránien d'une femelle teckel stérilisée de 11 ans. Le chien a été admis à la clinique en raison de toux, de régurgitations sporadiques et de dyspnée. L'échographie thoracique et la tomodensitométrie ont révélé une masse médiastinale importante qui a été retirée chirurgicalement par sternotomie. La tumeur était d'origine thymique et présentait 3 composantes distinctes : une composante épithéliale positive pour la pancytokératine (AE1/AE3) et la cytokératine de haut poids moléculaire (CK5/CK6) avec quelques espaces kystiques; un composant mésenchymateux positif à la vimentine; et en association avec la partie épithéliale, un composant mélanocytaire mineur positif pour Melan A. Des métastases histologiques des composants épithéliaux et mélanocytaires étaient présentes dans un ganglion lymphatique trachéobronchique. Le chien est décédé 105 jours après l'intervention chirurgicale, à la suite d'un épisode de dyspnée aiguë.Message clinique clé :À la connaissance des auteurs, il s'agit du premier cas de carcinosarcome thymique avec différenciation mélanocytaire.(Traduit par Dr Serge Messier).


Sujet(s)
Carcinosarcome , Maladies des chiens , Tumeurs du thymus , Animaux , Chiens , Maladies des chiens/anatomopathologie , Maladies des chiens/chirurgie , Maladies des chiens/diagnostic , Femelle , Carcinosarcome/médecine vétérinaire , Carcinosarcome/anatomopathologie , Carcinosarcome/chirurgie , Carcinosarcome/diagnostic , Tumeurs du thymus/médecine vétérinaire , Tumeurs du thymus/chirurgie , Tumeurs du thymus/anatomopathologie , Tumeurs du thymus/diagnostic , Issue fatale , Mélanocytes/anatomopathologie
2.
Medicine (Baltimore) ; 103(27): e38797, 2024 Jul 05.
Article de Anglais | MEDLINE | ID: mdl-38968487

RÉSUMÉ

RATIONALE: Pulmonary sarcomatoid carcinoma (PSC), a rare tumor, comprises 0.1% to 0.4% of all malignant lung tumors. Given the rarity of PSC, its clinical course, therapeutic guidelines, and patient outcomes remain largely unknown. Therefore, it is imperative to alert clinicians to this extremely rare and instructive early-onset cancer. PATIENT CONCERNS: This report describes a 28-year-old woman with PSC, who was initially misdiagnosed with Whipple's disease. A conclusive diagnosis of PSC was made following careful clinical examination, imaging, and histopathological evaluation of the patient's biopsy sample. Radiological imaging revealed multiple nodules and mass formations in the left upper lobe of the patient's lung, with the largest measuring of 5.4 × 3.2 cm. DIAGNOSIS: Histopathological examination indicated the presence of a malignant neoplasm associated with necrosis suggestive of sarcoma, which was pathologically staged as cT4N1M1. INTERVENTIONS AND OUTCOMES: A regimen of doxorubicin and ifosfamide was administered therapeutically, resulting in a stable disease state. LESSONS: The rarity and tumor origin challenge the diagnosis, which emphasizes the imperative role of histological examination, immunohistochemistry, and flow cytometry in achieving an accurate diagnosis. This report summarizes the existing publications to provide a comprehensive overview of PSC, including its clinical manifestations, radiographic imaging, pathologic features, diagnostic challenges, treatment strategies, and prognosis, and aims to improve the understanding of PSC.


Sujet(s)
Tumeurs du poumon , Humains , Femelle , Tumeurs du poumon/diagnostic , Tumeurs du poumon/anatomopathologie , Adulte , Diagnostic différentiel , Sarcomes/diagnostic , Sarcomes/anatomopathologie , Carcinosarcome/diagnostic , Carcinosarcome/anatomopathologie
3.
Medicine (Baltimore) ; 103(28): e38800, 2024 Jul 12.
Article de Anglais | MEDLINE | ID: mdl-38996113

RÉSUMÉ

RATIONALE: Uterine carcinosarcoma (UCS) is a rare and highly invasive malignant tumor.It exhibits an ectopic growth pattern of the uterus,and its histological features are biphasic differentiation of malignant epithelial components (cancer) and malignant mesenchymal components (sarcoma). The pathological pattern of high-component neuroendocrine differentiation is extremely rare. Due to the inherent heterogeneity of tumors, it increases the difficulty of accurate identification and diagnosis. The author introduces a rare case of primary endometrial carcinosarcoma (heterologous) with small cell neuroendocrine carcinoma (SCNEC) components. There is limited literature on this rare pathological differentiation pattern and a lack of guidelines for the best treatment methods, which prompts reflection on the diagnosis, optimal treatment strategies, and how preoperative diagnosis can affect patient prognosis for endometrial carcinosarcoma with neuroendocrine differentiation. PATIENT CONCERNS: The patient is an elderly woman who presents with abnormal vaginal bleeding after menopause. Transvaginal ultrasound examination shows that the uterus is slightly enlarged, and there is a lack of homogeneous echogenicity in the uterine cavity. Subsequently, a hysteroscopic curettage was performed, and a space-occupying lesion was observed on the anterior wall of the uterine cavity. DIAGNOSES: Preoperative endometrial biopsy revealed SCNEC of the endometrium. The patient underwent radical hysterectomy, and the postoperative pathological results showed that UCS (heterologous) was accompanied by SCNEC components (about 80%). INTERVENTION: The patient received radical hysterectomy, followed by adjuvant chemotherapy. OUTCOME: After 7 months of follow-up, no tumor recurrence or metastasis was found at the time of writing this article. LESSONS: The histological type of UCS (heterologous) with cell neuroendocrine carcinoma components is rare and highly invasive, with a high misdiagnosis rate in preoperative biopsy. There are currently no effective treatment guidelines for this type of case. The unusual appearance of SCNEC components in this case poses a challenge for both pathologists and surgeon. The rare differentiation pattern of this case exposes the complexity of its management and the necessity of prospective trials to determine the optimal treatment plan.


Sujet(s)
Carcinosarcome , Tumeurs de l'utérus , Humains , Femelle , Carcinosarcome/diagnostic , Carcinosarcome/anatomopathologie , Carcinosarcome/thérapie , Carcinosarcome/chirurgie , Tumeurs de l'utérus/anatomopathologie , Tumeurs de l'utérus/diagnostic , Tumeurs de l'utérus/chirurgie , Carcinome neuroendocrine/anatomopathologie , Carcinome neuroendocrine/diagnostic , Carcinome neuroendocrine/chirurgie , Sujet âgé , Hystérectomie/méthodes , Tumeurs de l'endomètre/anatomopathologie , Tumeurs de l'endomètre/diagnostic , Tumeurs de l'endomètre/thérapie
4.
Nihon Shokakibyo Gakkai Zasshi ; 121(6): 505-513, 2024.
Article de Japonais | MEDLINE | ID: mdl-38853020

RÉSUMÉ

A 68-year-old female patient was referred to our hospital with acute cholangitis. Computed tomography revealed common bile duct dilatation, gallbladder fundal tumor, and gallbladder wall thickening attached to the tumor. Cholangiography revealed pancreaticobiliary maljunction with biliary dilation. The patient was diagnosed with pancreaticobiliary maljunction with biliary dilation and gallbladder cancer and underwent liver S4b+5 and bile duct resection and reconstruction. Pathological results revealed that the gallbladder fundal tumor included sarcoma, and the gallbladder wall thickening had adenocarcinoma;thus, the patient was diagnosed with gallbladder carcinosarcoma.


Sujet(s)
Carcinosarcome , Tumeurs de la vésicule biliaire , Anomalie de jonction biliopancréatique , Humains , Femelle , Tumeurs de la vésicule biliaire/imagerie diagnostique , Tumeurs de la vésicule biliaire/chirurgie , Sujet âgé , Carcinosarcome/imagerie diagnostique , Carcinosarcome/chirurgie , Carcinosarcome/anatomopathologie , Anomalie de jonction biliopancréatique/imagerie diagnostique
5.
Chin Clin Oncol ; 13(3): 42, 2024 Jun.
Article de Anglais | MEDLINE | ID: mdl-38859609

RÉSUMÉ

BACKGROUND: Neutrophilia is an increase in the number of neutrophils over 7.5×103/µL. An increase in leukocytes over 50×103/µL is called a leukemoid reaction; and when it is associated with a solid tumor, it is considered a paraneoplastic syndrome called paraneoplastic leukemoid reaction (PLR). It is a very rare clinical condition and it is very unusual for it to be associated with carcinosarcoma. We present two cases of a leukemoid reaction observed in the Medical Oncology Department of the University Hospital of Salamanca between May and September 2023. The main objectives of our article are to describe the unusual appearance of paraneoplastic leukocytosis at the diagnosis of carcinosarcoma carcinosarcoma, explain in a detailed way its diagnostic procedure and to show the poor prognosis to which it is associated. CASE DESCRIPTION: In our presentation, we describe two similar cases: first of all, a 60-year-old woman without relevant medical history. She was referred by her primary physician to the Department of Internal Medicine in August 2023 with asthenia, lumbar pain, and weight loss of 12 kg of 3 months of evolution. The physical examination revealed a palpable hypogastric mass. An abdominal, pelvic, and thoracic computed tomography (CT) scan revealed a heterogenous solid mass with necrotic areas originating in the uterus. The anatomopathological diagnosis was carcinosarcoma. The patient showed a progressive worsening in her renal function associated with hyperviscosity secondary to hyperleukocytosis caused by 170×103/µL neutrophils. In the second case we describe the diagnosis of a PLR secondary to a kidney carcinosarcoma. When the patient started chemotherapy, he presented 55.08×103/µL leukocytes, 53.16×103/µL neutrophils. Eight days after receiving chemotherapy, the patient was admitted as an emergency with oligoanuria and decreased consciousness. He presented creatinine 6.25 mg/dL, phosphate 12.4 mg/dL, leukocytes 1.05×103/µL, and neutrophils 0.71×103/µL. The clinical diagnosis was acute exacerbation of multifactorial mixed (renal and prerenal) chronic kidney disease associated with tumor lysis syndrome and grade 3 neutropenia. The patient presented a poor evolution, dying after 2 months. CONCLUSIONS: PLR is a severe paraneoplastic syndrome associated with different types of solid tumors. Its appearance at the time of diagnosis of a tumor implies a poor vital prognosis.


Sujet(s)
Carcinosarcome , Hyperleucocytose , Syndromes paranéoplasiques , Humains , Carcinosarcome/complications , Femelle , Adulte d'âge moyen , Hyperleucocytose/étiologie , Hyperleucocytose/complications , Syndromes paranéoplasiques/étiologie , Mâle
6.
Vestn Otorinolaringol ; 89(2): 105-108, 2024.
Article de Russe | MEDLINE | ID: mdl-38805472

RÉSUMÉ

A clinical case of a rare malignant tumor of the larynx, carcinosarcoma, is described. The features of the clinical picture, diagnostic methods: instrumental and morphological are presented.


Sujet(s)
Carcinosarcome , Tumeurs du larynx , Humains , Carcinosarcome/diagnostic , Carcinosarcome/anatomopathologie , Diagnostic différentiel , Tumeurs du larynx/diagnostic , Tumeurs du larynx/anatomopathologie , Laryngectomie/méthodes , Laryngoscopie/méthodes , Larynx/anatomopathologie
7.
Am J Surg Pathol ; 48(8): 953-964, 2024 Aug 01.
Article de Anglais | MEDLINE | ID: mdl-38780000

RÉSUMÉ

Since the establishment of 4 molecular subgroups of endometrial carcinoma (EC), there has been significant interest in understanding molecular classification in the context of histologic features and diagnoses. ECs with undifferentiated, spindle, and/or sarcomatous components represent a diagnostically challenging subset of tumors with overlapping clinical and histologic features. We examined the clinicopathologic, morphologic, immunohistochemical, and molecular features of these tumors identified in our institutions' pathology databases using immunohistochemistry and targeted sequencing. Disease-specific survival (DSS) and progression-free survival (PFS) were analyzed using Kaplan-Meier curves and log-rank tests. One hundred sixty-two ECs were included: carcinosarcomas (UCS; n=96), dedifferentiated/undifferentiated EC (DDEC/UDEC; n=49), and grade 3 endometrioid EC with spindled growth (GR3spEEC) (n=17). All molecular subgroups were represented in all histologic subtypes and included 12 (7%) POLE -mutated ( POLE mut), 43 (27%) mismatch repair-deficient (MMRd), 77 (48%) p53-abnormal (p53abn), and 30 (19%) no specific molecular profile (NSMP) tumors. However, the molecular classification (irrespective of histologic diagnosis) was a significant predictor for both DSS ( P =0.008) and P≤0.0001). POLE mut EC showed an excellent prognosis with no recurrences or deaths from the disease. MMRd tumors also showed better outcomes relative to NSMP and p53abn tumors. In conclusion, molecular classification provides better prognostic information than histologic diagnosis for high-grade EC with undifferentiated and sarcomatous components. Our study strongly supports routine molecular classification of these tumors, with emphasis on molecular group, rather than histologic subtyping, in providing prognostication.


Sujet(s)
Marqueurs biologiques tumoraux , Tumeurs de l'endomètre , Grading des tumeurs , Humains , Femelle , Tumeurs de l'endomètre/anatomopathologie , Tumeurs de l'endomètre/classification , Tumeurs de l'endomètre/mortalité , Tumeurs de l'endomètre/génétique , Sujet âgé , Adulte d'âge moyen , Marqueurs biologiques tumoraux/analyse , Marqueurs biologiques tumoraux/génétique , Sujet âgé de 80 ans ou plus , Immunohistochimie , Survie sans progression , Carcinosarcome/anatomopathologie , Carcinosarcome/mortalité , Carcinosarcome/classification , Carcinosarcome/génétique , Adulte , Valeur prédictive des tests , Différenciation cellulaire , Carcinome endométrioïde/anatomopathologie , Carcinome endométrioïde/classification , Carcinome endométrioïde/mortalité , Carcinome endométrioïde/génétique , Mutation , Études rétrospectives , Facteurs temps
8.
Article de Anglais | MEDLINE | ID: mdl-38760285

RÉSUMÉ

True malignant mixed tumors, also known as salivary gland carcinosarcoma (SCS), are uncommon yet highly aggressive lesions associated with a poor prognosis. These tumors exhibit a distinctive biphasic structure characterized by both epithelial and mesenchymal components. Recent research has shown that the majority of SCS cases stem from pre-existing pleomorphic adenomas (PAs), suggesting a stepwise developmental pattern. In this report, we present a case of a 73-year-old female with SCS and describe the clinical, radiographic, and pathologic observations. Notably, the SCS was associated with a residual PA. The SCS displayed a CTNNB1::PLAG1 gene rearrangement, providing a molecular basis for its origin from the PA. Further DNA genomic analysis exposed mutations in BAP1, PER1, and LRPB1. Our findings provide support to the theory that SCS emerges from a pre-existing PA while highlighting the multiple genetic changes that could contribute to malignant transformation.


Sujet(s)
Adénome pléomorphe , Carcinosarcome , Humains , Femelle , Sujet âgé , Carcinosarcome/génétique , Carcinosarcome/anatomopathologie , Adénome pléomorphe/génétique , Adénome pléomorphe/anatomopathologie , Adénome pléomorphe/imagerie diagnostique , Tumeurs des glandes salivaires/génétique , Tumeurs des glandes salivaires/anatomopathologie , Tumeurs des glandes salivaires/imagerie diagnostique
9.
Head Neck Pathol ; 18(1): 44, 2024 May 22.
Article de Anglais | MEDLINE | ID: mdl-38775845

RÉSUMÉ

While acinic cell carcinoma (AciCC) can undergo high-grade transformation (HGT) to high-grade adenocarcinoma or poorly differentiated carcinoma, other morphologies such as spindle cell/sarcomatoid carcinoma are rare and not well-characterized. We herein report a novel case of AciCC with squamoglandular and chondrosarcomatous HGT mimicking a so-called 'carcinosarcoma ex-pleomorphic adenoma'. The patient is an 81-year-old male with a two-month history of neck swelling and referred otalgia who presented with a left parapharyngeal space mass extending into retropharyngeal space and pterygoid muscles. On resection, the tumor showed considerable morphologic diversity with high-grade serous and mucous acinar components as well as cribriform to solid apocrine-like components with comedonecrosis and squamous differentiation, all of which were embedded in a chondromyxoid background ranging from paucicellular and bland to a high-grade chondrosarcoma/pleomorphic sarcoma-like appearance. Only a minor conventional AciCC component was noted. Immunostains were negative for AR and only focally positive for GCDFP-15 arguing against a true apocrine phenotype, while PLAG1 and HMGA2 were negative arguing against an antecedent pleomorphic adenoma. On the other hand, SOX-10, DOG-1 and PAS after diastase highlighted serous acinar differentiation, and mucicarmine, and NKX3.1 highlighted mucous acinar differentiation. NR4A3 immunohistochemical staining and NR4A3 fluorescence in situ hybridization were positive in the carcinomatous and sarcomatoid components while sequencing analysis of both components revealed identical alterations involving TP53, PIK3CB, ARID1A, and STK11. This unique case warrants caution in designating all salivary sarcomatoid carcinomas with heterologous elements as part of the 'carcinoma ex-pleomorphic adenoma' family.


Sujet(s)
Adénome pléomorphe , Carcinome à cellules acineuses , Tumeurs des glandes salivaires , Humains , Mâle , Sujet âgé de 80 ans ou plus , Diagnostic différentiel , Carcinome à cellules acineuses/anatomopathologie , Carcinome à cellules acineuses/diagnostic , Adénome pléomorphe/anatomopathologie , Adénome pléomorphe/diagnostic , Tumeurs des glandes salivaires/anatomopathologie , Tumeurs des glandes salivaires/diagnostic , Carcinosarcome/anatomopathologie , Transformation cellulaire néoplasique/anatomopathologie , Terminologie comme sujet , Chondrosarcome/anatomopathologie , Chondrosarcome/diagnostic
10.
Medicine (Baltimore) ; 103(19): e38147, 2024 May 10.
Article de Anglais | MEDLINE | ID: mdl-38728484

RÉSUMÉ

RATIONALE: Sarcomatoid carcinoma of the small intestine is an exceedingly rare and aggressive malignancy, often diagnosed at advanced stages with a poor prognosis. This study documents a detailed case of sarcomatoid carcinoma of the small intestine, highlighting the diagnostic challenges and treatment approaches, underscored by a comprehensive review of related literature. Given the rarity of this condition, our report aims to enrich the existing diagnostic and treatment frameworks for this malignancy, emphasizing the necessity for early detection and intervention strategies. By presenting this case in conjunction with a literature review, we seek to shed light on the elusive nature of sarcomatoid carcinoma in the small intestine and propose avenues for improving patient outcomes. PATIENT CONCERNS: Case presentation A 61-year-old male patient initially presented with recurrent abdominal pain and gastrointestinal symptoms. Initial abdominal computed tomography (CT) scans and gastrointestinal endoscopy revealed only inflammatory and hyperplastic changes in the duodenum and jejunum, with a diagnosis of intestinal obstruction. Two years later, due to gastrointestinal perforation, the patient was hospitalized again. DIAGNOSES: CT scans and other examinations revealed small intestinal lesions. Four small intestinal lesions were surgically removed, and pathology and immunohistochemistry confirmed sarcomatoid carcinoma of the small intestine. A short time later, enhanced CT scans revealed metastatic lesions in the hepatic portal and adrenal glands. INTERVENTIONS: After surgery, the gastrointestinal function gradually recovered, and the patient was discharged from the hospital on a semiliquid diet. No further treatment such as radiotherapy or chemotherapy was administered postoperatively. OUTCOMES: Five months after the surgery, the patient died due to brain metastasis. LESSONS: The study outcomes reveal the aggressive nature of sarcomatoid carcinoma of the small intestine, characterized by rapid progression and poor prognosis despite surgical interventions. The patient condition rapidly deteriorated, leading to metastasis and death within 5 months postsurgery. These findings underscore the critical need for early detection and possibly innovative treatment approaches to improve survival rates. This case also highlights the potential for gastrointestinal sarcomatoid carcinoma to metastasize to distant organs, including the brain, suggesting a propensity for hematogenous spread.


Sujet(s)
Perforation intestinale , Humains , Mâle , Adulte d'âge moyen , Perforation intestinale/étiologie , Perforation intestinale/chirurgie , Intestin grêle/anatomopathologie , Tumeurs de l'intestin/anatomopathologie , Tumeurs de l'intestin/complications , Carcinosarcome/anatomopathologie , Carcinosarcome/diagnostic , Carcinosarcome/complications , Tomodensitométrie
12.
Medicina (B Aires) ; 84(2): 337-341, 2024.
Article de Espagnol | MEDLINE | ID: mdl-38683520

RÉSUMÉ

Primary hepatic sarcomatoid carcinoma is a very aggressive tumor, representing 0.4-0.7% of all primary hepatic neoplasms. The disease is associated with liver disease due to hepatotropic viruses and is more prevalent in Asians. Histology shows sarcomatous and carcinoma components. It does not have pathognomonic clinical or imaging characteristics and its diagnosis is based on the pathological and immunohistochemistry findings. Surgery could prolong survival in localized stages. We report the case of a 72-year-old Korean patient with a history of chronic liver disease due to B virus, who was diagnosed with primary hepatic sarcomatoid carcinoma with bone and lymph node metastases.


El carcinoma sarcomatoide primario hepático es un tumor agresivo que representa el 0.4-0.7% de todas las neoplasias primarias hepáticas. Se asocia a hepatopatía por virus hepatotropos, es más prevalente en la población asiática y en su histología se evidencian componentes de carcinoma y sarcoma. No posee características clínicas ni imagenológicas patognomónicas y su diagnóstico se realiza en base a los hallazgos de la anatomía patológica e inmunohistoquímica. La cirugía en estadio localizado representa la única modalidad terapéutica con impacto en la sobrevida. Reportamos el caso de una paciente de 72 años, coreana, con antecedentes de hepatopatía crónica por virus B, a quien se le diagnosticó un carcinoma sarcomatoide hepático primario con metástasis ósea y ganglionares.


Sujet(s)
Tumeurs du foie , Humains , Sujet âgé , Tumeurs du foie/anatomopathologie , Tumeurs du foie/imagerie diagnostique , Mâle , Métastase lymphatique/anatomopathologie , Carcinosarcome/anatomopathologie , Carcinosarcome/imagerie diagnostique
13.
Clin. transl. oncol. (Print) ; 26(4): 1033-1037, Abr. 2024.
Article de Anglais | IBECS | ID: ibc-VR-66

RÉSUMÉ

Objective: To know the risk of endometrial cancer (EC) in a population of women with BRCA 1/2 pathogenic or likely pathogenic variants after risk-reducing salpingo-oophorectomy (RRSO). Methods: The study cohort included data from 857 women with BRCA mutations who underwent RRSO visited four hospitals in Catalonia, Spain, from January 1, 1999 to April 30, 2019. Standardized incidence ratio (SIR) of EC was calculated in these patients using data from a regional population-based cancer registry. Results: After RRSO, eight cases of EC were identified. Four in BRCA 1 carriers and four in BRCA2 carriers. The expected number of cases of EC was 3.67 cases, with a SIR of 2.18 and a 95% CI (0.93–3.95). Conclusions: In our cohort, the risk of EC in BRCA1/2 carriers after RRSO is not greater than expected. Hysterectomy is not routinely recommended for these patients.(AU)


Sujet(s)
Humains , Mâle , Femelle , Carcinome endométrioïde , Carcinosarcome , Hystérectomie , Tumeurs de l'endomètre , Tumeurs du sein , Salpingo-ovariectomie , Études de cohortes , Mutation , Tamoxifène , Prédisposition génétique à une maladie
14.
World J Surg Oncol ; 22(1): 104, 2024 Apr 18.
Article de Anglais | MEDLINE | ID: mdl-38637834

RÉSUMÉ

OBJECTIVES: To investigate the clinicopathological characteristics and prognosis of patients with primary sarcoma of the uterine cervix. METHODS: We identified all patients with primary cervical sarcomas treated at our institution from 2002 to 2020 and analyzed the clinicopathological characteristics and prognosis. RESULTS: 34 patients were identified, 7 (20.6%) patients had leiomyosarcoma, 6 (17.6%) had carcinosarcoma, 5 (14.7%) had Ewing sarcoma, 4 (11.8%) had rhabdomyosarcoma, 4 (11.8%) had undifferentiated sarcoma, 2 (5.9%) had adenosarcoma, 2 (5.9%) had endometrial stromal sarcoma, 1 (2.9%) had dermatofibrosarcoma protuberans, 1 (2.9%) had alveolar soft tissue sarcoma and 2 (5.9%) had sarcoma not otherwise specified. The median age of the whole patients was 43.5 years (range, 13-63). The median age of patients with Ewing sarcoma or rhabdomyosarcoma was 22 years (range, 13-39) and 17 years (range, 13-36 years), respectively. The distribution by stage was: stage I in 21 (61.8%) patients, stage II in 4 (11.8%), stage III in 6 (17.6%) and stage IV in 3 (8.8%). Overall, 30 patients (88.2%) received surgical treatment. The median follow-up was 33.3 months (range 3.6-187.3 months). 11 patients died within 2 years after diagnosis, most of them were patients with carcinosarcoma or undifferentiated sarcoma (45.5%, 5/11). In the entire cohort, 2- and 5-year OS were 67.2% and 56.9%, respectively. 5-year OS was 25.0% for undifferentiated sarcoma, 50.0% for rhabdomyosarcoma, 50.0% for carcinosarcoma, 53.3% for Ewing sarcoma, 57.1% for leiomyosarcoma. CONCLUSION: Cervical sarcomas are rare neoplasms with multiple histological subtypes and follow an aggressive course. Prognosis may be associated with tumor histology and stage.


Sujet(s)
Carcinosarcome , Léiomyosarcome , Rhabdomyosarcome , Sarcome d'Ewing , Sarcomes , Tumeurs du col de l'utérus , Tumeurs de l'utérus , Femelle , Humains , Adolescent , Jeune adulte , Adulte , Adulte d'âge moyen , Léiomyosarcome/anatomopathologie , Sarcome d'Ewing/chirurgie , Tumeurs du col de l'utérus/chirurgie , Tumeurs de l'utérus/diagnostic , Sarcomes/chirurgie , Sarcomes/diagnostic , Carcinosarcome/anatomopathologie , Rhabdomyosarcome/chirurgie , Pronostic
15.
Front Immunol ; 15: 1374270, 2024.
Article de Anglais | MEDLINE | ID: mdl-38650938

RÉSUMÉ

Pulmonary sarcomatoid carcinoma (PSC) represents a rare and highly aggressive variant of lung cancer, characterized by its recalcitrance to conventional therapeutic modalities and the attendant dismal prognosis it confers. Recent breakthroughs in immunotherapy have presented novel prospects for PSC patients; nevertheless, the utility of neoadjuvant/conversional immunotherapy in the context of PSC remains ambiguous. In this report, we present a middle-aged male presenting with Stage III PSC, notable for its high expression of the programmed death-ligand 1 (PD-L1), initially deemed as non-resectable for sizeable tumor mass and multiple lymph nodes metastases. The patient underwent a transformation to a resectable state after a regimen of three cycles of platinum-based chemotherapy plus immunotherapy. Following definitive surgical resection, the individual realized a pathological complete response (pCR), culminating in a significant prolongation of event-free survival (EFS). This case underscores the viability of employing immunochemotherapy as a neoadjuvant/conversional strategy for chosen cases of PSC.


Sujet(s)
Tumeurs du poumon , Humains , Mâle , Tumeurs du poumon/thérapie , Tumeurs du poumon/traitement médicamenteux , Tumeurs du poumon/anatomopathologie , Adulte d'âge moyen , Stadification tumorale , Protocoles de polychimiothérapie antinéoplasique/usage thérapeutique , Traitement néoadjuvant/méthodes , Immunothérapie/méthodes , Résultat thérapeutique , Antigène CD274/antagonistes et inhibiteurs , Carcinosarcome/thérapie , Carcinosarcome/anatomopathologie , Carcinosarcome/traitement médicamenteux
16.
Clin Oncol (R Coll Radiol) ; 36(6): e137-e145, 2024 Jun.
Article de Anglais | MEDLINE | ID: mdl-38565457

RÉSUMÉ

AIMS: Sinonasal teratocarcinosarcomas (SNTCS) are rare sinonasal malignancies, the incidence of which is less than 1% of all tumors. There is limited data available on SNTCS's, often as case reports and small case series. The management of SNTCS is complicated because of its location, locally aggressive biology, difficulty in achieving complete resection, and limited data on chemotherapy in these malignancies. This audit was performed to understand the role of neoadjuvant chemotherapy (NACT) in SNTCS's, its ability to downstage the disease, achieve complete resection, and impact on long-term survival outcomes. METHODS: This was a retrospective analysis of a prospectively maintained database approved by the Institutional Ethics Committee (IEC). The baseline characteristics, the extent of tumor, Kadish stage, NACT regimen, and adverse events were extracted from the Electronic Medical Records and the patient's case file. Patients with baseline extensive/inoperable disease were referred for NACT from the multidisciplinary joint clinic followed by response assessment (RECIST v1.1). Patients underwent skull-base surgery if respectable post-completion of NACT, however, if deemed unresectable were treated with non-surgical modalities or palliative therapies. RESULTS: The data of 27 patients were evaluated from the year 2015-2022. The median age was 42 years (IQR:30-56) and 85.2% (n = 23) were males. The ECOG-PS was 0-1 in 88.8% (n = 24) patients. All 27 patients received NACT in view of extensive disease at presentation. 74.1% (n = 20) patients received Cisplatin-Etoposide and 25.9% (n = 7) received other chemotherapy regimens. The median number of chemotherapy cycles was 2(IQR:2-3). 96.3% patients (n = 26) completed the planned NACT cycles. 70.4% (n = 19) patients achieved a partial response in post-NACT imaging. 77.8% (n = 18) underwent surgery, 18.5% (n = 5) received CTRT, and 7.4% (n = 2) received definitive-RT alone. The median PFS and OS of the cohort was 19months (95%CI:12.0-25.6) and 23months (95%CI:5.94-40.06) respectively. CONCLUSION: NACT is safe, feasible, and effective with significant response rates, leading to effective downstaging, resectability and improved survival in patients with locally advanced SNTCS's.


Sujet(s)
Carcinosarcome , Traitement néoadjuvant , Tumeurs du nez , Centres de soins tertiaires , Humains , Mâle , Femelle , Études rétrospectives , Inde , Adulte , Adulte d'âge moyen , Traitement néoadjuvant/méthodes , Carcinosarcome/traitement médicamenteux , Carcinosarcome/thérapie , Carcinosarcome/anatomopathologie , Tumeurs des sinus de la face/traitement médicamenteux , Tumeurs des sinus de la face/thérapie , Tumeurs des sinus de la face/anatomopathologie , Tératome/traitement médicamenteux , Tératome/thérapie , Protocoles de polychimiothérapie antinéoplasique/usage thérapeutique , Sujet âgé , Traitement médicamenteux adjuvant/méthodes
17.
Asian J Surg ; 47(6): 2954-2955, 2024 Jun.
Article de Anglais | MEDLINE | ID: mdl-38433082

RÉSUMÉ

Hepatic sarcomatoid carcinoma (HSC) is a rare and highly aggressive liver malignancy. This report presents the case of a 62-year-old patient with chronic hepatitis B who presented with a 10 cm liver mass. The patient underwent a right hepatectomy, and pathological results confirmed the presence of HSC. Despite receiving comprehensive treatment, the patient had multiple abdominal lymph node metastases within 6 months after the operation, ultimately the patient passed away 10 months after the operation due to tumor progression.


Sujet(s)
Hépatectomie , Tumeurs du foie , Humains , Adulte d'âge moyen , Tumeurs du foie/chirurgie , Tumeurs du foie/anatomopathologie , Hépatectomie/méthodes , Mâle , Hépatite B chronique/complications , Issue fatale , Métastase lymphatique , Carcinosarcome/anatomopathologie , Carcinosarcome/chirurgie , Carcinosarcome/diagnostic , Tomodensitométrie , Évolution de la maladie
18.
Acta Cytol ; 68(2): 107-120, 2024.
Article de Anglais | MEDLINE | ID: mdl-38437817

RÉSUMÉ

INTRODUCTION: Poorly differentiated primary sarcomatoid parotid malignancies are extremely rare. These tumors have not been consistently studied by morphology, immunohistochemistry, or molecular techniques. CASE PRESENTATION: We report three unusual cases of parotid gland poorly-differentiated sarcomatoid malignancy investigated by fine-needle aspiration and studied histologically, by immunohistochemistry and molecular investigations. Aspirates showed poorly specific polymorphous sarcomatoid malignancy in all cases. Histologically, all cases were polymorphous high-grade malignancies, and additionally, one case showed epithelial structures and was finally classified as salivary carcinosarcoma. Immunohistochemistry showed classical melanocytic markers negativity but positivity for PRAME, CD10, and WT1 in all three tumors and for CD56 in two tumors, which can potentially be supportive of melanocytic origin. Although not entirely specific, molecular characterization also suggested the melanocytic lineage of these tumors. CONCLUSION: Although rare, primary malignant melanoma of salivary gland was already described, but undifferentiated/dedifferentiated amelanotic forms are unknown in this localization up today. Further case reports of similar presentations are required to confirm the unequivocal primary origin of these obscure neoplasms in the parotid gland.


Sujet(s)
Marqueurs biologiques tumoraux , Immunohistochimie , Mélanome , Tumeurs de la parotide , Adulte , Sujet âgé , Femelle , Humains , Mâle , Marqueurs biologiques tumoraux/analyse , Marqueurs biologiques tumoraux/génétique , Cytoponction , Carcinosarcome/anatomopathologie , Carcinosarcome/diagnostic , Différenciation cellulaire , Mélanome/anatomopathologie , Mélanome/diagnostic , Glande parotide/anatomopathologie , Tumeurs de la parotide/anatomopathologie , Tumeurs de la parotide/diagnostic
19.
Zhongguo Fei Ai Za Zhi ; 27(2): 157-160, 2024 Feb 20.
Article de Chinois | MEDLINE | ID: mdl-38453449

RÉSUMÉ

Pulmonary sarcomatoid carcinoma (PSC) is a rare and highly malignant tumor, which includes the following five pathologic types: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma and pulmonary blastoma. The onset of PSC is occult with non-specific clinical symptoms and signs. The clinical manifestations include irritating cough, bloody sputum, dyspnea, chest pain and so on, which are closely related to the growth and invasion site of the tumor. PSC tends to metastasize early, so most patients are already in local advanced stage or advanced stage with a median survival of 9 months at the time of hospital visit. A patient with primary PSC which led to 90% stenosis in central airway was treated by combined method of vascular and tracheoscopic intervention in our respiratory center. This treatment prolonged the patient's survival time and got a satisfactory effect at 19-month follow-up after surgery. Herein we report the case for clinical reference.
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Sujet(s)
Carcinomes , Carcinosarcome , Tumeurs du poumon , Humains , Tumeurs du poumon/diagnostic , Pronostic , Carcinomes/anatomopathologie , Carcinosarcome/chirurgie , Carcinosarcome/anatomopathologie , Poumon/anatomopathologie
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