RESUMEN
A relatively healthy male patient in his 60s presented with chest pain and shortness of breath in addition to a history of significant weight loss over the preceding months. He was admitted to the hospital and investigated with a CT pulmonary angiogram, which did not demonstrate a pulmonary embolus, but he subsequently went on to have an ultrasound and CT scan because of abnormal findings. His CT demonstrated some thickening of the mid-transverse colon, and, in addition, large volume liver metastases described as innumerable and probably replacing most of the liver.Initially, his liver function tests were only mildly deranged at the presentation. Flexible sigmoidoscopy was performed, and a transverse colonic malignancy was identified and biopsied, which demonstrated an extrapulmonary small cell carcinoma (EPSCC). He was admitted for urgent chemotherapy for newly diagnosed metastatic small-cell colonic cancer; he developed tumour lysis syndrome following his first dose of chemotherapy. He continued to decline following this and died soon after his admission. Metastatic small-cell colonic cancer is a rare diagnosis which is challenging to manage due to the lack of trial evidence to drive treatment strategies. The management largely follows the pulmonary small cell cancer pathway. We, therefore, present a colonic EPSCC case outlining the diagnostic and treatment strategies for this disease.
Asunto(s)
Carcinoma de Células Pequeñas , Neoplasias Hepáticas , Humanos , Masculino , Resultado Fatal , Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/patología , Carcinoma de Células Pequeñas/patología , Carcinoma de Células Pequeñas/diagnóstico , Carcinoma de Células Pequeñas/secundario , Carcinoma de Células Pequeñas/complicaciones , Persona de Mediana Edad , Tomografía Computarizada por Rayos X , Neoplasias Pulmonares/patología , Neoplasias Pulmonares/secundario , Neoplasias Pulmonares/diagnóstico , Neoplasias Colorrectales/patología , Neoplasias del Colon/patologíaRESUMEN
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Asunto(s)
Carcinoma de Células Pequeñas , Hipercalcemia , Neoplasias Ováricas , Humanos , Femenino , Hipercalcemia/diagnóstico por imagen , Neoplasias Ováricas/diagnóstico por imagen , Neoplasias Ováricas/complicaciones , Carcinoma de Células Pequeñas/diagnóstico por imagen , Carcinoma de Células Pequeñas/complicaciones , Ultrasonografía/métodos , Diagnóstico Diferencial , Persona de Mediana EdadRESUMEN
INTRODUCTION: Lurbinectedin is FDA approved for treatment of metastatic small cell lung cancer (SCLC) following progression on or after platinum-based chemotherapy. Prostatic small cell or neuroendocrine carcinoma (SC/NEPC) behaves like SCLC; however, no safety or efficacy data for lurbinectedin in SC/NEPC exists. PATIENTS AND METHODS: All SC/NEPC patients treated with lurbinectedin across 4 academic oncology centers were identified. Baseline patient data and lurbinectedin outcomes including radiographic responses (complete response [CR], partial response [PR], stable disease [SD], progressive disease [PD]), progression free survival (PFS), overall survival (OS), and treatment-related adverse events (trAEs) were described. Clinical benefit rate (CBR) included CR, PR, or SD on imaging. Descriptive statistics were performed. RESULTS: At first lurbinectedin dose, all 18 patients had metastatic disease. Median age was 63.5 (Range: 53-84), number of prior systemic therapies was 4 (Range: 2-7), and lurbinectedin cycles completed was 5 (Range: 1-10). ADT was administered during lurbinectedin treatment in 9/18 patients. CBR was 9/16 (56%). The most common trAEs were fatigue and anemia. Median OS and PFS were 6.01 (0.23-16.69) and 3.35 (0.16-7.79) months. CONCLUSIONS: Lurbinectedin showed modest but significant clinical benefit in some patients with SC/NEPC and demonstrated an acceptable toxicity profile with no hospitalizations from trAEs. SC/NEPC is an aggressive disease with a poor prognosis for which more treatment options are needed. Evidence for subsequent treatments after platinum-based chemotherapy is lacking. Lurbinectedin is an active treatment option for SC/NEPC; however, larger confirmatory studies are needed.
Asunto(s)
Carbolinas , Carcinoma Neuroendocrino , Compuestos Heterocíclicos de 4 o más Anillos , Neoplasias de la Próstata , Humanos , Masculino , Carbolinas/administración & dosificación , Carbolinas/uso terapéutico , Carbolinas/efectos adversos , Anciano , Carcinoma Neuroendocrino/tratamiento farmacológico , Carcinoma Neuroendocrino/patología , Compuestos Heterocíclicos de 4 o más Anillos/uso terapéutico , Compuestos Heterocíclicos de 4 o más Anillos/administración & dosificación , Compuestos Heterocíclicos de 4 o más Anillos/efectos adversos , Persona de Mediana Edad , Anciano de 80 o más Años , Neoplasias de la Próstata/tratamiento farmacológico , Neoplasias de la Próstata/patología , Resultado del Tratamiento , Carcinoma de Células Pequeñas/tratamiento farmacológico , Carcinoma de Células Pequeñas/patología , Antineoplásicos/uso terapéutico , Antineoplásicos/administración & dosificación , Antineoplásicos/efectos adversos , Estudios Retrospectivos , Supervivencia sin ProgresiónRESUMEN
RATIONALE: Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a rare and aggressive gynecological tumor. We retrospectively analyzed the clinical manifestations and imaging findings of this patient and analyzed the relevant literature, with the aim of improving the ability of radiologists to differentiate SCCOHT from other ovarian tumors. PATIENT CONCERNS: We report a case of 36-year-old woman who was diagnosed with SCCOHT. MRI suggested a malignant tumor of the left ovary. The immunohistochemical markers shows SMARCA4 negativity. Notably, hypercalcemia was not detected. Microscopically, it was consistent with the large-cell variants. LESSIONS: Despite its rarity, SCCOHT should still be considered in the differential diagnosis of ovarian malignancies. When a young female patient presents with a large unilateral tumor on MRI with a predominant solid component and significant enhancement on the contrast enhanced scans, along with hypercalcemia, SCCOHT should be considered.
Asunto(s)
Carcinoma de Células Pequeñas , Hipercalcemia , Imagen por Resonancia Magnética , Neoplasias Ováricas , Humanos , Femenino , Neoplasias Ováricas/diagnóstico por imagen , Neoplasias Ováricas/patología , Neoplasias Ováricas/diagnóstico , Adulto , Hipercalcemia/etiología , Hipercalcemia/diagnóstico por imagen , Carcinoma de Células Pequeñas/diagnóstico por imagen , Carcinoma de Células Pequeñas/diagnóstico , Imagen por Resonancia Magnética/métodos , Diagnóstico DiferencialAsunto(s)
Carcinoma Neuroendocrino , Carcinoma de Células Escamosas , Neoplasias Hipofaríngeas , Humanos , Femenino , Neoplasias Hipofaríngeas/patología , Persona de Mediana Edad , Carcinoma de Células Escamosas/patología , Carcinoma Neuroendocrino/patología , Carcinoma de Células Pequeñas/terapia , Carcinoma de Células Pequeñas/patologíaRESUMEN
ABSTRACT: Postpneumonectomy syndrome (PPS) is a rare, life-threatening complication characterized by dynamic airway obstruction due to mediastinal rotation at any time point following pneumonectomy. This can produce life-threatening respiratory and cardiovascular complications. We report a case who developed PPS following right pneumonectomy in a 55-year-old female patient with small cell carcinoma (SCC) right lung.
Asunto(s)
Obstrucción de las Vías Aéreas , Neoplasias Pulmonares , Neumonectomía , Complicaciones Posoperatorias , Carcinoma Pulmonar de Células Pequeñas , Humanos , Femenino , Neumonectomía/efectos adversos , Persona de Mediana Edad , Neoplasias Pulmonares/cirugía , Complicaciones Posoperatorias/etiología , Síndrome , Obstrucción de las Vías Aéreas/etiología , Obstrucción de las Vías Aéreas/cirugía , Carcinoma Pulmonar de Células Pequeñas/cirugía , Carcinoma Pulmonar de Células Pequeñas/complicaciones , Carcinoma de Células Pequeñas/cirugía , Carcinoma de Células Pequeñas/complicaciones , Tomografía Computarizada por Rayos XRESUMEN
Small cell carcinoma of the bladder (SCCB) is a rare cancer that accounts for approximately 1% of primary malignant bladder tumors. It is highly malignant and has a poor prognosis. Similar to small cell lung cancer, platinum-based chemotherapy is recommended as the first-line therapy, and amrubicin (AMR) is recommended as the second-line therapy, but there is no established therapy after the second line. We report a case of SCCB that was refractory to multiple chemotherapies but responded to pembrolizumab. A 77-year-old male, diagnosed with clinical stage T3N0M0 small cell carcinoma and invasive urothelial carcinoma by transurethral resection of bladder tumor (TURBT), underwent robot-assisted radical cystectomy after three cycles of neoadjuvant cisplatin-irinotecan chemotherapy, and pathological examination revealed only small cell carcinoma in his cystectomy specimen. After three courses of adjuvant carboplatin-etoposide chemotherapy, the patient developed liver and bone metastases. Furthermore, after two courses of amrubicin, we started pembrolizumab due to the progression of metastases. Metastases decreased after starting pembrolizumab and continued to decrease after discontinuation because of immunerelated adverse events (irAEs). Therefore, pembrolizumab may be an option for the treatment of refractory SCCB.
Asunto(s)
Anticuerpos Monoclonales Humanizados , Carcinoma de Células Pequeñas , Neoplasias de la Vejiga Urinaria , Humanos , Masculino , Neoplasias de la Vejiga Urinaria/tratamiento farmacológico , Neoplasias de la Vejiga Urinaria/patología , Neoplasias de la Vejiga Urinaria/cirugía , Anciano , Anticuerpos Monoclonales Humanizados/uso terapéutico , Carcinoma de Células Pequeñas/tratamiento farmacológico , Carcinoma de Células Pequeñas/cirugía , Carcinoma de Células Pequeñas/diagnóstico por imagen , Resultado del Tratamiento , Antineoplásicos Inmunológicos/uso terapéutico , CistectomíaRESUMEN
We report a case of small cell carcinoma of the urethra with inguinal lymph node metastases. A 50- year-old female patient presented with gross hematuria. Cystoscopy and computed tomography (CT) revealed a tumor surrounding the urethra and an inguinal lymphadenopathy. Biopsy of the urethral tumor demonstrated small cell carcinoma. Four courses of chemotherapy with etoposide and cisplatin, followed by 66 Gy of irradiation achieved complete remission. Unfortunately, 14 months later, positroemission-CT scan revealed recurrence of inguinal lymph node metastases. Although seven courses of chemotherapy with nogitecan were carried out, a new metastatic bone tumor developed. Amrubicin was administered as a third-line treatment, but was canceled after one course because of side effects. The patient died at 39 months after diagnosis. Small cell carcinoma of urethra with metastases has extremely poor prognosis, as is demonstrated by this case.
Asunto(s)
Carcinoma de Células Pequeñas , Neoplasias Uretrales , Humanos , Femenino , Persona de Mediana Edad , Carcinoma de Células Pequeñas/diagnóstico por imagen , Carcinoma de Células Pequeñas/terapia , Carcinoma de Células Pequeñas/patología , Neoplasias Uretrales/patología , Neoplasias Uretrales/diagnóstico por imagen , Neoplasias Uretrales/terapia , Metástasis Linfática , Resultado Fatal , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Esophageal cancer is quite prevalent worldwide and usually carries a poor prognosis. Histologically, although squamous cell carcinoma and adenocarcinoma predominate, small cell carcinoma (SmCC) cases have been reported. Overall, there is a paucity of literature regarding this variant. In this article, we aim to highlight this uncommon entity of carcinoma esophagus and share our experience of SmCC patients seen over a decade at our institute. METHOD: Records of patients with SmCC histology from 2010 to 2020 were assessed. Patients' demographic characteristics, clinical characteristics, treatment received, and outcomes were taken into consideration. Results were analyzed statistically using SPSS version 22. RESULTS: Fourteen patients (nine males and five females) with a median age of 57 years (range: 35 - 72 years) were analyzed. The majority of the patients 10/14 (71.4%) received palliative radiotherapy of either 30Gy/10 fractions or 35Gy/15 fractions. Only 1/14 (7.14%) patients received neoadjuvant chemotherapy and concurrent chemoradiation (CCRT). Overall, partial response was noted in all 11 patients (78.6%) who received treatment. The average median survival was 5 months (range: 1-11 months). CONCLUSION: Although the small sample size of the study prevents us from drawing a firm conclusion, we propose national and international collaborative prospective studies for framing definitive oncologic management strategies for this rare histological variant of esophageal cancer.
Asunto(s)
Carcinoma de Células Pequeñas , Neoplasias Esofágicas , Centros de Atención Terciaria , Humanos , Masculino , Neoplasias Esofágicas/patología , Neoplasias Esofágicas/terapia , Neoplasias Esofágicas/mortalidad , Neoplasias Esofágicas/diagnóstico , Femenino , Persona de Mediana Edad , Adulto , Anciano , Carcinoma de Células Pequeñas/terapia , Carcinoma de Células Pequeñas/patología , Carcinoma de Células Pequeñas/mortalidad , Centros de Atención Terciaria/estadística & datos numéricos , Pronóstico , Quimioradioterapia/métodos , Estudios Retrospectivos , Tasa de Supervivencia , Terapia Neoadyuvante/métodos , Resultado del Tratamiento , EsofagectomíaRESUMEN
OBJECTIVE: To explore the clinical manifestations, diagnosis, pathological features and treatment of small-cell carcinoma of the prostate (SCCP). METHODS: We conducted a retrospective analysis of the clinical and pathological data of 2 cases of confirmed SCCP treated from November 2017 to March 2018, and reviewed relevant literature. RESULTS: Both the patients had the symptoms of frequent, urgent and difficult urination, with an elevated level of PSA and gradesâ ¡ï¼â ¢ enlargement of the prostate at palpation. One underwent prostate puncture biopsy and the other received transurethral 1470 laser vaporization resection of the tumor. Postoperative pathology indicated prostate adenocarcinoma accompanied by SCCP in both of the cases. One of them was treated by etoposide-platinum (EP) chemotherapy and died of systemic multiple organ failure 20 months after diagnosis, while the other underwent endocrine therapy and has lived with tumor up to the present day. CONCLUSION: The incidence rate of SCCP is low, its malignancy is high, and its prognosis is poor. The average survival of the patient is about 7 to 10 months after diagnosis. Currently there is no effective management of the dissease, except by relying on the experience of the treatment of small-cell lung cancer, with chemotherapy as the main option.
Asunto(s)
Carcinoma de Células Pequeñas , Neoplasias de la Próstata , Humanos , Masculino , Carcinoma de Células Pequeñas/diagnóstico , Carcinoma de Células Pequeñas/terapia , Neoplasias de la Próstata/diagnóstico , Neoplasias de la Próstata/terapia , Neoplasias de la Próstata/patología , Estudios Retrospectivos , Etopósido/uso terapéutico , Etopósido/administración & dosificación , Anciano , Persona de Mediana Edad , Próstata/patología , Pronóstico , Adenocarcinoma/diagnóstico , Adenocarcinoma/patología , Adenocarcinoma/terapia , Antígeno Prostático Específico/sangreRESUMEN
BACKGROUND: There is currently scarcity of information on small cell neuroendocrine carcinoma of the nasopharynx (SCNEC-nasopharynx). It is believed that this type of cancer is not associated with Epstein-Barr virus (EBV) infection and is indistinguishable from classic SCNEC occurring in other organs. MATERIALS AND METHODS: Herein we provided 3 cases of nasopharyngeal mass in our hospital, two males and one female. On admission, these patients were considered nasopharyngeal carcinoma with lymph node metastasis, and one of them had liver metastasis. The nasopharyngeal mucosal tissues were biopsied for pathological examination including immunohistochemistry and in situ hybridization. PubMed database was searched for articles about SCNEC-nasopharynx published up to April 2024 in any language. RESULT: The 3 cases had similar histological features of SCNEC in other organs but differed in rich- tumor-infiltrating lymphocytes (TILs). All of them stained for pancytokeratin (panCK) and epidermal growth factor receptor (EGFR). Case 1 and Case 2 diffusely expressed insulinoma-associated protein 1(INSM-1) and synaptophysin (Syn), Case 3 strongly stained for CD56 and Syn. Immunostaining of all 3 cases for p40, p63, TTF-1, CK20, S-100 and NUT showed negative. BRG-1, INI-1 and Rb were retained. And p53 all showed wild-type expression. The Ki-67 labeling indiced of case 1, 2, and 3 were 80%, 90%, and 80%, respectively. In situ hybridization showed strong and uniform nuclear positivity of EBV-encoded small RNAs (EBER) in the neoplastic cells of 3 cases. CONCLUSION: EBV-positive SCNEC-nasopharynx was exactly rare. The origin of this tumor is still controversial. It may originate from EBV-infected mucosal epithelium like nasopharyngeal carcinoma. Based on our cases and relevant literature, we found EBV-positive SCNEC-nasopharynx as a probably site-specific subtype of SCNEC with differing pathogenetic mechanism. The subtype not only virus positivity but also that it was associated with TILs and did not show p53 or Rb alterations by immunohistochemistry. It may be more responsive to treatment and have a better prognosis than classic SCNEC. We will continue to follow-up these patients and collect additional cases to further understand the unique biology of this rare solid tumor.
Asunto(s)
Carcinoma Neuroendocrino , Infecciones por Virus de Epstein-Barr , Neoplasias Nasofaríngeas , Humanos , Masculino , Femenino , Carcinoma Neuroendocrino/virología , Carcinoma Neuroendocrino/patología , Neoplasias Nasofaríngeas/virología , Neoplasias Nasofaríngeas/patología , Persona de Mediana Edad , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/patología , Infecciones por Virus de Epstein-Barr/virología , Carcinoma Nasofaríngeo/virología , Carcinoma Nasofaríngeo/patología , Inmunohistoquímica , Biomarcadores de Tumor/análisis , Herpesvirus Humano 4/aislamiento & purificación , Herpesvirus Humano 4/genética , Carcinoma de Células Pequeñas/virología , Carcinoma de Células Pequeñas/patología , Carcinoma de Células Pequeñas/química , Adulto , AncianoRESUMEN
Small cell carcinoma of the esophagus (SCCE) is a rare and highly malignant type of esophageal cancer with no standard treatment, facing challenges of resistance to conventional therapies. This study presents the cases of one extensive-stage and two limited-stage SCCE patients treated with chemoimmunotherapy. The two limited-stage patients underwent surgery post-treatment and experienced notable and enduring positive responses. This represents the first documented application of neoadjuvant chemoimmunotherapy in limited-stage SCCE patients. Additionally, comprehensive immunohistochemical analysis and whole exome sequencing were performed on the case patients. The findings revealed that infiltration of CD8+ T cells and PD-L1 expression in the SCCE tumor were key factors for favorable responses in SCCE patients receiving chemoimmunotherapy.
Asunto(s)
Carcinoma de Células Pequeñas , Neoplasias Esofágicas , Inmunoterapia , Terapia Neoadyuvante , Humanos , Neoplasias Esofágicas/terapia , Neoplasias Esofágicas/tratamiento farmacológico , Terapia Neoadyuvante/métodos , Carcinoma de Células Pequeñas/terapia , Carcinoma de Células Pequeñas/tratamiento farmacológico , Masculino , Inmunoterapia/métodos , Persona de Mediana Edad , Antígeno B7-H1/metabolismo , Resultado del Tratamiento , Anciano , Biomarcadores de Tumor , Linfocitos T CD8-positivos/inmunología , Femenino , Secuenciación del ExomaRESUMEN
Presentamos el caso de un paciente con antecedentes de hipertensión arterial vasculorrenal tratada un año antes, que acude a urgencias por emergencia hipertensiva (HTA) y disnea. Descartada primera sospecha de reestenosis de arteria renal con angiografía por tomografía computarizada (angioTC), se completa el estudio confirmándose diagnóstico de cáncer de pulmón mediante prueba de imagen y anatomía patológica. En el estudio de hipertensión se detecta elevación de hormona adrenocorticótropa (ACTH), hipercortisolismo y datos analíticos de hiperaldosteronismo. Con el diagnóstico final de síndrome de Cushing secundario a producción ectópica de ACTH se inicia tratamiento médico, sin llegar a recibir nada más por fallecimiento del paciente a los pocos días.(AU)
We present the case of a patient with a history of renal-vascular hypertension treated with stent one year previously, who attended the emergency room due to hypertensive emergency and dyspnea. Once the first suspicion of renal artery restenosis was ruled out with CT angiography, the study was completed, confirming the diagnosis of lung cancer through imaging and pathological anatomy. In the hormonal study, elevation of ACTH, hypercortisolism and analytical data of hyperaldosteronism were detected. With the final diagnosis of Cushing's syndrome secondary to ectopic production of ACTH, medical treatment was started, without being able to receive anything else due to the death of the patient after a few days.(AU)
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Síndrome de Cushing , Hipertensión , Carcinoma de Células Pequeñas , Neoplasias Pulmonares , Hiperaldosteronismo , Alcalosis , Pacientes Internos , Examen Físico , Enfermedades Cardiovasculares , NefrologíaRESUMEN
Neuroendocrine carcinoma arising from the urachus is extremely rare. We describe a case of a 33-year-old gentleman who presented with hematuria and diagnosed to have a composite adenocarcinoma and small cell neuroendocrine carcinoma arising from the urachus. The patient also had widespread metastasis at the time of presentation, therefore, he was referred for chemotherapy. However, the disease showed progression despite treatment. Recognition of neuroendocrine carcinoma component in urachal tumors, although rare, is very essential as this histologic type carries poor prognosis with aggressive clinical outcome.
Asunto(s)
Adenocarcinoma , Carcinoma Neuroendocrino , Resistencia a Antineoplásicos , Neoplasias de la Vejiga Urinaria , Humanos , Masculino , Adulto , Carcinoma Neuroendocrino/tratamiento farmacológico , Carcinoma Neuroendocrino/patología , Carcinoma Neuroendocrino/secundario , Neoplasias de la Vejiga Urinaria/patología , Neoplasias de la Vejiga Urinaria/tratamiento farmacológico , Adenocarcinoma/tratamiento farmacológico , Adenocarcinoma/secundario , Adenocarcinoma/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Resultado Fatal , Neoplasias Complejas y Mixtas/patología , Neoplasias Complejas y Mixtas/tratamiento farmacológico , Carcinoma de Células Pequeñas/tratamiento farmacológico , Carcinoma de Células Pequeñas/patología , Carcinoma de Células Pequeñas/secundarioRESUMEN
Small cell bladder cancer (SCBC) is a rare and aggressive disease, often treated with platinum/etoposide-based chemotherapy. Key molecular drivers include the inactivation of onco-suppressor genes (TP53, RB1) and amplifications in proto-oncogenes (MYC). We report a patient with SCBC who achieved an objective and prolonged response to lurbinectedin, which has been approved for metastatic small cell lung cancer, after developing disease progression on cisplatin/etoposide and nivolumab/ipilimumab. A genomic analysis of a metastatic biopsy prior to lurbinectedin initiation revealed a TP53 mutation and amplification of the cell cycle regulators E2F3 and MYCL. A repeat biopsy following the development of lurbinectedin resistance showed a new actionable ERBB2 alteration without significant change in the tumor mutation burden (six mutations/Mb). The present report suggests that lurbinectedin may be active and should be further explored in SCBC harboring TP53 mutations and amplifications in E2F3 and MYC family complexes.
Asunto(s)
Carbolinas , Compuestos Heterocíclicos de 4 o más Anillos , Mutación , Proteína p53 Supresora de Tumor , Neoplasias de la Vejiga Urinaria , Humanos , Carbolinas/uso terapéutico , Neoplasias de la Vejiga Urinaria/tratamiento farmacológico , Neoplasias de la Vejiga Urinaria/genética , Neoplasias de la Vejiga Urinaria/patología , Compuestos Heterocíclicos de 4 o más Anillos/uso terapéutico , Compuestos Heterocíclicos de 4 o más Anillos/farmacología , Proteína p53 Supresora de Tumor/genética , Masculino , Carcinoma de Células Pequeñas/tratamiento farmacológico , Carcinoma de Células Pequeñas/genética , Compuestos Heterocíclicos con 3 Anillos/uso terapéutico , Antineoplásicos/uso terapéutico , Persona de Mediana EdadRESUMEN
BACKGROUND Small cell carcinoma is an aggressive malignant neuroendocrine tumor that most commonly occurs in the lung. Primary small cell carcinoma of the esophagus (PSCCE) is rare and is an aggressive malignancy with poor prognosis and no clear management guidelines. This report describes the case of a 36-year-old man presenting with epigastric pain, dysphagia, and melena due to a primary esophageal small cell carcinoma. CASE REPORT A 36-year-old presented to the Emergency Department (ED) with epigastric pain associated with food intake. Initial workup was unremarkable, and a presumed clinical diagnosis of reflux esophagitis and peptic strictures was made, prompting empiric treatment with anti-secretory therapies. Despite these therapies, he presented to the emergency room with progressively worsening dysphagia. Endoscopic examination (EGD) revealed a large necrotic mass, and computed tomography (CT) imaging revealed liver metastasis. Biopsies from both the liver and esophageal masses confirmed small cell carcinoma. His clinical course was complicated by a broncho-esophageal fistula, leading to massive hemoptysis, necessitating intubation. Unfortunately, his condition deteriorated rapidly, and he chose to pursue hospice care. He died 3 months after his initial presentation. CONCLUSIONS This report has presented a rare case of primary esophageal small cell carcinoma and our approach to management. We highlight the importance of early diagnosis, supported by histopathology, and the need for management guidelines.
Asunto(s)
Dolor Abdominal , Carcinoma de Células Pequeñas , Trastornos de Deglución , Neoplasias Esofágicas , Humanos , Masculino , Adulto , Trastornos de Deglución/etiología , Carcinoma de Células Pequeñas/complicaciones , Carcinoma de Células Pequeñas/diagnóstico , Neoplasias Esofágicas/complicaciones , Neoplasias Esofágicas/diagnóstico , Resultado Fatal , Dolor Abdominal/etiología , Neoplasias Hepáticas/complicaciones , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/secundario , Tomografía Computarizada por Rayos XAsunto(s)
Anticuerpos Monoclonales Humanizados , Protocolos de Quimioterapia Combinada Antineoplásica , Bevacizumab , Carcinoma de Células Pequeñas , Ciclofosfamida , Neoplasias Ováricas , Humanos , Femenino , Bevacizumab/administración & dosificación , Ciclofosfamida/administración & dosificación , Anticuerpos Monoclonales Humanizados/administración & dosificación , Anticuerpos Monoclonales Humanizados/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Ováricas/tratamiento farmacológico , Neoplasias Ováricas/patología , Carcinoma de Células Pequeñas/tratamiento farmacológico , Carcinoma de Células Pequeñas/patología , Hipercalcemia/tratamiento farmacológico , Persona de Mediana EdadRESUMEN
Small cell cervical carcinoma (SCCC) is the most common neuroendocrine tumor in the female genital tract, with an unfavorable prognosis and lacking an evidence-based therapeutic approach. Until now, the distinct subtypes and immune characteristics of SCCC combined with genome and transcriptome have not been described. We performed genomic (n = 18), HPV integration (n = 18), and transcriptomic sequencing (n = 19) of SCCC samples. We assessed differences in immune characteristics between SCCC and conventional cervical cancer, and other small cell neuroendocrine carcinomas, through bioinformatics analysis and immunohistochemical assays. We stratified SCCC patients through non-negative matrix factorization and described the characteristics of these distinct types. We further validated it using multiplex immunofluorescence (n = 77) and investigated its clinical prognostic effect. We confirmed a high frequency of PIK3CA and TP53 alterations and HPV18 integrations in SCCC. SCCC and other small cell carcinoma had similar expression signatures and immune cell infiltration patterns. Comparing patients with SCCC to those with conventional cervical cancer, the former presented immune excluded or 'desert' infiltration. The number of CD8+ cells in the invasion margin of SCCC patients predicted favorable clinical outcomes. We identified three transcriptome subtypes: an inflamed phenotype with high-level expression of genes related to the MHC-II complex (CD74) and IFN-α/ß (SCCC-I), and two neuroendocrine subtypes with high-level expression of ASCL1 or NEUROD1, respectively. Combined with multiple technologies, we found that the neuroendocrine groups had more TP53 mutations and SCCC-I had more PIK3CA mutations. Multiplex immunofluorescence validated these subtypes and SCCC-I was an independent prognostic factor of overall survival. These results provide insights into SCCC tumor heterogeneity and potential therapies. © 2024 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.
Asunto(s)
Carcinoma Neuroendocrino , Microambiente Tumoral , Neoplasias del Cuello Uterino , Humanos , Femenino , Neoplasias del Cuello Uterino/genética , Neoplasias del Cuello Uterino/inmunología , Neoplasias del Cuello Uterino/patología , Neoplasias del Cuello Uterino/virología , Microambiente Tumoral/inmunología , Microambiente Tumoral/genética , Carcinoma Neuroendocrino/genética , Carcinoma Neuroendocrino/inmunología , Carcinoma Neuroendocrino/patología , Carcinoma Neuroendocrino/mortalidad , Carcinoma de Células Pequeñas/genética , Carcinoma de Células Pequeñas/inmunología , Carcinoma de Células Pequeñas/patología , Persona de Mediana Edad , Biomarcadores de Tumor/genética , Adulto , Mutación , Transcriptoma , Fosfatidilinositol 3-Quinasa Clase I/genética , Pronóstico , Perfilación de la Expresión Génica/métodos , Anciano , MultiómicaRESUMEN
Small-cell neuroendocrine carcinoma of the cervix (SCNECC) is a rare cancer with poor prognosis, with limited data to guide its treatment. The objective of this study was to evaluate practice patterns in the management of SCNECC. A 23-question online survey on management of SCNECC was disseminated to Canadian gynecologic oncologists (GO), radiation oncologists (RO) and medical oncologists (MO). In total, 34 practitioners from eight provinces responded, including 17 GO, 13 RO and four MO. During staging and diagnosis, 74% of respondents used a trimodality imaging approach, and 85% tested for neuroendocrine markers. In early-stage (1A1-1B2) SCNECC, 87% of practitioners used a surgical-based approach with various adjuvant and neoadjuvant treatments. In locally advanced (1B3-IVA) SCNECC, 53% favored primary chemoradiation, with cisplatin and etoposide, with the remainder using surgical or radiation-based approaches. In metastatic and recurrent SCNECC, the most common first-line regimen was etoposide and platinum, and 63% of practitioners considered clinical trials in the first line setting or beyond. This survey highlights diverse practice patterns in the treatment of SCNECC. Interdisciplinary input is crucial to individualizing multimodality treatment, and there is a need for prospective trials and intergroup collaboration to define the optimal approach towards managing this rare cancer type.