Carcinoma escamoso variedad sarcomatoide de lengua. Reporte de caso / Sarcomatoid variant squamous cell carcinoma of the tongue. Case report

Introducción. El carcinoma de células escamosas es una patología relativamente frecuente en Paraguay, que debe ser diagnosticada y tratada a tiempo. La variedad sarcomatoide es un subtipo poco frecuente, pero mucho más agresivo que la presentación convencional, con altas tasas de recurrencia y metástasis linfática. La exposición previa a radiación es uno de los principales factores desencadenantes. Caso clínico. Paciente de 83 años con antecedente de radioterapia por carcinoma escamocelular del paladar blando, quien consultó por una masa en el borde lateral de la lengua que correspondió a un carcinoma escamocelular del subtipo sarcomatoide. Resultados. El paciente fue sometido a cirugía y quimioterapia, pero presentó recaída tumoral a los cuatro meses, sin aceptar una cirugía de rescate, optando por el tratamiento paliativo y falleciendo a los pocos meses. Conclusión. El examen exhaustivo de la cavidad oral en una primera consulta permite identificar lesiones en estadios tempranos y el tratamiento multidisciplinario temprano puede mejorar la supervivencia global. El pronóstico de estos pacientes en estadios avanzados es desalentador. Actualmente la cirugía microvascular es la mejor opción terapéutica, pero la hemiglosectomía sin reconstrucción sigue siendo una opción aceptable en nuestro medio, conociendo los altos costos de la primera y el requerimiento de un grupo mayor de especialistas, largos tiempos quirúrgicos y estancias hospitalarias.

Introduction. Squamous cell carcinoma is a relatively common pathology in Paraguay, which must be diagnosed and treated on time. The sarcomatoid variety is a rare subtype, but much more aggressive than the conventional presentation, with high rates of recurrence and lymphatic metastasis. Previous exposure to radiation is one of the main triggering factors. Clinical case. An 83-year-old patient with a history of radiotherapy for squamous cell carcinoma of the soft palate, who consulted for a mass on the lateral edge of the tongue that corresponded to a squamous cell carcinoma of the sarcomatoid subtype. Results. The patient underwent surgery and chemotherapy, but had tumor relapse after four months, without accepting salvage surgery, opting for palliative treatment and dying a few months later. Conclusion. Exhaustive examination of the oral cavity in a first consultation allows lesions to be identified in early stages and early multidisciplinary treatment can improve overall survival. The prognosis of these patients in advanced stages is discouraging. Currently, microvascular surgery is the best therapeutic option, but hemiglossectomy without reconstruction continues to be an acceptable option in our environment, knowing the high costs of the former and the requirement for a larger group of specialists, long surgical times and hospital stays.

Management challenges of a cervical carcinosarcoma in a premenopausal woman in northern Tanzania: A rare case report and review of current literature.

INTRODUCTION AND IMPORTANCE: Cervical carcinosarcomas (CCS) are uncommon aggressive gynecological malignancies that typically occur in postmenopausal females. Data on CCS, incidence, clinical presentation, magnitude and management strategies remain scarce especially in Sub-Saharan countries like Tanzania. In this report, we report the rarity of this particular condition in premenopausal woman, the literature surrounding similar reports, and the many challenges that arise in the management of CCS. CASE PRESENTATION: We present a redudancy case of a 35-year-old female from Northern Tanzania who presented with vaginal bleeding, foul smelling discharge, and severe abdominal pain intermittently for a year. The Speculum exam revealed a fungating mass on the cervix, and CT confirmed the irregular mass centered in the uterine cervix expanding in the fornices infiltrating the uterine neck. A cervical biopsy revealed carcinosarcoma of the cervix stage IIA. Due to the patient's instability, she was admitted and started on chemotherapy and later on recommended for radiation without surgical intervention. CLINICAL DISCUSSION: In this case report, we discuss the condition's rarity and multiple barriers we encountered during management of this complicated patient with CCS. Choosing an optimal treatment approach was challenging due to the lack of guidelines and available literature as well as the patient's instability. CONCLUSION: his case is notable due to the rarity of this cervical neoplasm especially in premenopausal women and the challenging management of the condition due to a notable lack of treatment recommendations.

Initial Presentation of Ovarian Carcinosarcoma With Non-islet Cell Tumor Hypoglycemia: A Case Report.

Ovarian cancer, although not among the most commonly diagnosed cancers, remains a significant cause of cancer-related mortality in females. Several paraneoplastic syndromes have been associated, and this case study represents a rare manifestation of ovarian cancer, presenting as non-islet cell tumor hypoglycemia (NICTH), characterized by the excessive production of insulin-like growth factor-II (IGF-II) by tumor cells. We report a 55-year-old woman who presented to our hospital with abdominal distension and severe refractory hypoglycemia. The laboratory data revealed the suppression of serum insulin and C-peptide levels. The insulin-like growth factor II (IGF-II)/insulin-like growth factor 1 (IGF1) ratio was >32. The hypoglycemia was hence attributed to the non-islet cell tumor type, and it is likely driven by tumoral secretion of incompletely processed IGF-II. The lab findings suggested the existence of NICTH. Abdominal computed tomography demonstrated the presence of a left ovarian mass and peritoneal carcinomatosis. CT-guided biopsy of the peritoneal lesions showed poorly differentiated malignancy consistent with ovarian carcinosarcoma (OCS). The patient was treated with a continuous infusion of glucose. She even received oral prednisone and glucagon infusion. Chemotherapy with carboplatin and paclitaxel was initiated, but unfortunately, she died from complications of multiorgan failure. To our knowledge, this is the first novel case of an initial presentation of metastatic OCS with NICTH, underscoring the complexity of ovarian cancer presentations and the necessity of a comprehensive approach in managing rare paraneoplastic syndromes, such as NICTH.

Ovarian carcinosarcomas: p53 status defines two distinct patterns of oncogenesis and outcomes.

Objectives: Ovarian carcinosarcoma (OCS) is a rare and lethal type of ovarian cancer. Despite its incredibly poor prognosis, it has received little research attention. In this study, we aim to evaluate the molecular features of OCS and elucidate their clinical significance. Study methods: We examined 30 OCS by immunohistochemistry (IHC) and targeted panel sequencing collected from a single institution (2003-2013) as the initial molecularly characterized cohort (Cohort A). From November 2016 to April 2023, we collected an additional 67 OCS cases from three institutions across British Columbia and Alberta as the contemporary cohort (Cohort B) for clinical correlation. The Kaplan-Meier method was used to estimate overall and progression-free survival, and differences in survival rates were compared using the log-rank test. All tests were two-sided. A p-value of less than 0.05 was considered statistically significant. Results: The majority of OCS (82%) in the initial Cohort A were p53-mutated, and the carcinomatous component displayed the histological and molecular features of a high-grade tubo-ovarian serous carcinoma (HGSC-like). In a minority of OCS, the epithelial components were characteristics of endometrioid or clear cell carcinomas, and IHC staining was wild type for p53. In the contemporary Cohort B, we observed the same histological findings related to the p53 IHC staining pattern. The median overall survival of the p53-mutated HGSC-like OCS (47 patients) was significantly higher (43.5 months) compared with that of the p53 wild-type OCS (10 patients, 8.8 months; P < 0.01). Pathogenic BRCA1/2 germline/somatic mutations were observed in 7 patients (17.5%) of HGSC-like OCS, and all these patients were alive at 3 years from diagnosis compared to a 51% 3-year survival among the patients with BRCA1/2 wild-type HGSC-like OCS (33 patients) (p = 0.022). Majority of patients (6/7) with BRCA1/2-mutated OCS received poly (ADP-ribose) polymerase inhibitor as maintenance therapy in this cohort. Conclusions: Most OCSs have a morphologic and molecular profile resembling HGSC; however, some OCSs display a molecular profile that suggests origin through non-serous oncogenic pathways. This molecular distinction has both prognostic and treatment (predictive) implications. These findings underscore the importance of routine p53 IHC testing on all OCS and BRCA1/2 testing on p53-mutated OCS.

Strategic multidisciplinary management of pelvic carcinosarcoma: Emphasizing advanced diagnostic imaging and staged surgical interventions.

Pelvic carcinosarcoma is an aggressive malignancy with significant diagnostic and management hurdles due to its complex vascularity and potential for extensive local invasion. A 59-year-old female presented with severe abdominal pain and significant weight loss, leading to the discovery of a large, complex pelvic mass through CT scans, MRI, and PET CT, suggesting aggressive malignancy. Initial management included a robotic laparoscopic proximal sigmoid loop colostomy to alleviate obstruction. Significant vascularity led to consultations with Vascular Surgery and subsequent preoperative embolization. Definitive surgery involved a supralevator posterior exenteration for en bloc resection of the vagina, mass, and sigmoid colon, combined with a low anterior resection and an omental J flap in anticipation of potential postoperative radiation therapy. This case underscores the importance of integrated imaging and staged surgical interventions in managing pelvic carcinosarcoma, emphasizing a multidisciplinary approach to optimize outcomes and minimize complications.

The efficacy and safety of lenvatinib plus pembrolizumab therapy in patients with uterine carcinosarcoma.

Lenvatinib plus pembrolizumab (LP) therapy is currently used in patients with advanced or recurrent endometrial cancer. However, patients with uterine carcinosarcoma (UCS) were not included in the KEYNOTE-775, and the efficacy of LP therapy for patients with UCS in clinical practice remains unclear. We administered LP therapy to five patients with UCS. We aimed to report our clinical experience with LP therapy in these patients and investigate the genomic characteristics of those who responded to LP therapy. We retrospectively reviewed patients with UCS (n = 5) who underwent LP therapy at our hospital from January 2019 to December 2023. Efficacy was assessed using the response rate according to the Response Evaluation Criteria in Solid Tumors version 1.1. Safety was evaluated in terms of adverse events. The median age was 65 (55-78) years, and the mismatch repair status was proficient in all of the patients. One patient had stage II disease, and four had stage III. The median number of LP therapy courses was 8 (1-35). The overall response rate was 40%. None of the patients experienced adverse events that were grade 3 or higher. The median follow-up duration was 9 (1-26) months, median progression-free survival was 9.1 (0.16 to NA) months, and median overall survival was 10.2 (1.41 to NA) months. LP therapy may be effective for patients with UCS. As this report was based on a limited number of patients, more cases are required to investigate the efficacy of LP therapy in patients with UCS.

Clinical and Morphological Aspects of Aggressive Salivary Gland Mixed Tumors: A Narrative Review.

Salivary gland tumors are a rare and heterogeneous group of neoplasms of the head and neck region. The mixed category of these tumors include the following entities: pleomorphic adenoma (PA), carcinoma ex pleomorphic adenoma (CEPA), salivary carcinosarcoma (CS), and metastasizing PA (MPA). The most common benign tumor of the salivary glands is PA. Metastasis and malignant degeneration have been reported in cases of PA of a salivary gland origin. Judging by their behavior, MPA, CEPA, and CS can be considered aggressive tumors. Invasive CEPA has been identified in the parotid gland more frequently. MPA and CS cases reported in the current literature are rare. In this paper, we present, narratively, the clinico-morphological features of this group of mixed tumors.

Spotlight on Carcinosarcoma of the Ovary: A Scoping Review.

Ovarian carcinosarcoma, also referred as malignant mixed Mullerian tumour, is an uncommon, highly aggressive and malignant neoplasm which makes up 1 to 4% of all ovarian tumours. It is biphasic involving both malignant sarcomatous (mesenchymal) and carcinomatous (epithelial) cells. There are various subtypes such as serous and endometrioid. However, the mesenchymal part is sarcomatous. About 90% of cases of ovarian carcinosarcoma spread outside the ovary. The two most accepted theories of origin for carcinosarcoma of the ovary are the collision and conversion theories. A third theory is the combination theory. Prognosis remains poor even when still localised in the ovary. In the last few years, there has been no change in the survival rate. The median survival rate is lower than 2 years. Clinical features mainly include lower abdominal pain and a palpable abdominal mass. Ovarian carcinosarcoma remains poorly understood and understudied. Being a rare tumour, elaborate therapeutic consensus is not available for ovarian carcinosarcoma. The main treatment involves cytoreductive surgery and then chemotherapy. The type of chemotherapy, role of radiotherapy and novel therapies need to be further studied. The main objective of this article is to review the current literature on carcinosarcoma of the ovary.

A Case Study of a High-Grade Serous Carcinoma of the Fallopian Tube Transformed into Carcinosarcoma at the Site of Peritoneal Dissemination With Immunohistological Evidence of an Epithelial-Mesenchymal Transition.

We report a patient in whom a primary high-grade serous carcinoma (HGSC) of the fallopian tube transformed into a carcinosarcoma at the site of peritoneal dissemination, and immunohistological analysis suggested the involvement of an epithelial-mesenchymal transition (EMT). The patient, a 70-year-old woman, had an abdominal mass palpated on admission, and a laparotomy was performed after a close examination. The resected right fallopian tube was cystically dilated, and a solid mass was observed in its lumen. The histological diagnosis was HGSC of the right fallopian tube with a papillary or complex tubular structure composed of tumor cells with marked nuclear irregularities. p53 was overexpressed, and no mesenchymal tumor component was observed. The resected left-sided abdominal mass of the omentum was a solid with a long diameter of 100 mm. Microscopically, the tumor exhibited a mixture of HGSC and high-grade sarcoma with nonspecific differentiation. Furthermore, a heterologous chondrosarcoma was subsequently observed from the high-grade sarcoma. The HGSC component was E-cadherin positive. The high-grade sarcoma component was positive for EMT-related proteins such as zinc finger E-box-binding homeobox 1 (ZEB1) and twist family bHLH transcription factor 1 (TWIST1). The chondrosarcoma component was ZEB1 positive and TWIST1 negative. p53 overexpression was found in all 3 components. The tumor of the omentum suggested that an EMT phenomenon was involved in the tumorigenesis. In this scenario, the primary HGSC of the fallopian tube with obvious invasion demonstrated that the conversion from carcinoma to sarcoma by EMT occurs only with peritoneal dissemination.