Paraneoplastic leukocytosis secondary to carcinosarcoma: a report of two cases and literature review.

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.

[Active surveillance for patients with localized prostate cáncer. Adherence and protocol drop analysis.] / La vigilancia activa en pacientes con cáncer de próstata localizado. Análisis de adherencia y salida del protocolo.

OBJECTIVE: VA is currently considered the treatment of choice for patients with low and very low risk prostate cancer. We analyzed the evolution of this treatment strategy in our series and adherence to the protocol. MATERIAL AND METHODS: Ambispective study of patients in VA in our center between 2014- 2019. 237 meet inclusion criteria, of which 142 (60%) have a minimum of 12 months of follow- up. Mean age: 68.5 (4678), median PSA 6.37 ng / ml (1-33). 229 (96.6%) are ISUP 1 and 8 (3.4%) ISUP 2. Objectives are proposed to assess our adherence to the protocol. Descriptive statistics are used to communicate the results. RESULTS: According to the classification by risk groups of the NCCN, 145 (61.2%), 49 (20.7%) and 42 (17.7%) were very low risk, low risk and favorable intermediate risk patients, respectively. The median of follow-up is 14 months (0-66). Of the patients with a minimum follow-up of 12 months, 107 (75.4%) were re-biopsied. 80 (33.8%) leave the protocol in these 5 years, 31.3% (25) by their own decision, 55% (44) due to medical criteria, and 11.3% (9) go to WW. After 5 years of follow-up, 99.2% of patients are still alive, 0.8% died of specific non-cancer causes. Of the objectives to assess adherence, 8 are achieved, 1 partially and 1 is not evaluable. CONCLUSIONS: VA in our center is already the treatment of choice for very low-risk patients, with a constant increase from year to year. Adherence to the protocol has been favorable during the period of time studied.

OBJETIVO: La VA se ha convertido en uno de los tratamientos de elección del CP localizado de bajo y muy bajo riesgo. Analizamos la evolución de esta estrategia de tratamiento en nuestra serie, así como la adherencia al protocolo.MATERIAL Y MÉTODOS: Estudio ambispectivo de los pacientes incluidos en VA en nuestro centro entre los años 2014-2019. 237 pacientes cumplen los criterios de inclusión en VA, de los cuales 142 (60%) tienen un seguimiento mínimo de 12 meses. Edad media: 68,5(46-78), mediana PSA 6,37 ng/ml (1-33). 229 pacientes (96,6%) son ISUP 1 y 8 (3,4%) ISUP 2. Se proponen unos objetivos para valorar nuestra adherencia al protocolo. Se utiliza estadística descriptiva y contraste de hipótesis para comunicar los resultados.RESULTADOS Y DISCUSIÓN: Atendiendo a la clasificación por grupos de riesgo de la NCCN, 145 (61,2%), 49 (20,7%) y 42 (17,7%) eran pacientes muy bajo riesgo, bajo riesgo y riesgo intermedio favorable respectivamente. El tiempo (mediana) de permanencia en el programa es de 14 meses (0-66). De los pacientes con un seguimiento mínimo de 12 meses, 107 (75,4%) son re ­ biopsiados. 80 pacientes (33,8%) salen del protocolo en estos 5 años, 31,3% (25) por decisión propia, 55% (44) por criterios médicos, y 11,3% (9) pasan a WW. Tras 5 años de seguimiento, el 99,2% de los pacientes continúan vivos, el 0,8% falleció por causas no cáncer específicas. De los objetivos para evaluar la adherencia, 8 de ellos se alcanzan, 1 parcialmente y 1 no es evaluable. CONCLUSIONES: La VA en nuestro centro constituye actualmente el tratamiento de elección para los pacientes con muy bajo riesgo. La adherencia al protocolo ha sido favorable durante el periodo de tiempo estudiado.

Renal Cell Carcinoma Associated With Xp11.2 Translocation/TFE3 Gene-fusion: A Long Response to mammalian target of rapamycin (mTOR) Inhibitors.

OBJECTIVE: To demonstrate that patients with Xp11.2/TFE3 gene-fusion translocation renal cell carcinoma (RCC), despite having an aggressive course in young adults, could have valid treatment options such as mammalian target of rapamycin (mTOR) inhibitors with good outcomes. Furthermore, to explain possible mechanisms of action of mTOR inhibitors in this type of RCC. MATERIALS AND METHODS: We report a case of a 44-year-old man who has been treated with everolimus for a Xp11.2 translocation/TFE3 gene-fusion RCC after 2 previous failed treatments with tyrosine kinase inhibitor. During the follow-up, we evaluated type and duration of response with everolimus. RESULTS: The patient obtained a long-lasting response of disease of 25 months with everolimus without any symptom. CONCLUSION: We believe that mTOR inhibitors could be a good line option treatment to consider for this type of patients.

Influence of secondary diagnoses in the development of urinary incontinence after radical prostatectomy.

OBJECTIVE: To study whether there are factors related to secondary diagnoses (SDg) present in patients with prostate cancer that influence the development of urinary incontinence after radical prostatectomy (RP). MATERIALS AND METHODS: A retrospective multicenter observational study was performed reviewing the medical records of 430 men who underwent RP due to organ-confined prostate cancer in 9 different hospitals. Two study groups were distinguished: Group A (GA): Patients without urinary incontinence after RP; Group B (GB): patients with any degree of post-surgical urinary incontinence. RESULTS: Average age at surgery was 63.42 years (range 45-73). 258 patients were continent after surgery and 172 patients complaint of any degree of incontinence after RP. A higher percentage of healthy patients was found in group A (continent after surgery) than in group B (p = 0.001). The most common SDg prior to surgery were hypertension, lower urinary tract symptoms, dyslipidemia, diabetes mellitus and erectile dysfunction, but none did show a greater trend towards post-surgical incontinence. CONCLUSIONS: A better health status prior to surgery is associated to a lower incidence of new-onset urinary incontinence after radical prostatectomy. However, no correlation was found between the most common medical disorders and the development of post-surgical urinary incontinence.

Influence of antiplatelet-anticoagulant drugs on the need of blood components transfusion after vesical transurethral resection.

AIMS: The effect of the antithrombotic preventive therapy on haemorrhage keeps uncertain. We investigate the influence of the antiplatelet and anticoagulant drugs (AP/AC drugs) on the transfusion requirement after vesical transurethral resection (VTUR). We also describe the epidemiology of the blood components transfusion in our department. MATERIALS AND METHODS: Retrospective observational study of a series of patients needing blood transfusion at the Urology Department between June 2010 and June 2013. Selection of 100 consecutive patients who were transfused after VTUR due to bladder transitional cell carcinoma (BTCC) (group A = GA). CONTROL GROUP: 100 consecutive patients who underwent VTUR due to BTCC and were not transfused (group B = GB). Transfusion criteria: Haemoglobin < 8 g/dl + anaemia symptoms. Age, gender, associated AP/AC treatment, secondary diagnoses, toxics, tumour stage and grade were analysed. RESULTS: 212 patients required transfusion of a blood component. 169 were men (79%) and 43 women (21%). Median age 77.59 years (SD 9.42, range 50-92). Secondary diagnoses: Diabetes Mellitus 64%, high blood pressure 77%, dyslipidemia 52%. 60% of patients were previously treated with AP/AC drugs. Average Haemoglobin pre-transfusion values: 7.4 g/dl (DE ± 0.7). Average Haemoglobin post-transfusion values: 8.9 g/Dl (DE ± 0.72). Most frequent transfusion indications were bladder cancer (37%), kidney cancer (11%), prostate cancer (8%), benign prostatic hyperplasia (BHP) (8%), other urological diagnoses (36%). Intraoperative transfusions indicated by the anaesthesiologist: kidney cancer (33%), BPH (28%). Patients who underwent VTUR due to BTCC were older in GA (77.59 years SD 9.42) than in GB (68.98 years SD 11.78) (p = 0.0001). Similar gender distribution (15 women in GA and 24 in GB). Less patients were asked to keep their treatment with ASA 100mg (AcetylSalicylicAcid) in GA (25.64%) than in GB (50%) (p = 0.0330). More aggressive tumour grade in GA (p = 0.0003) and higher stage in GA (p = 0.0018) regardless of concomitant treatment with AP/AC drugs. CONCLUSIONS: The pathologies which most needed blood components' transfusions in the Urology Department were (in order of frequency): bladder cancer, kidney cancer, prostate cancer, prostate adenoma. ASA100mg did not influence the transfusion's requirements in VTUR due to BTCC. Tumour stage and higher grade have a greater influence in transfusion's requirements than concomitant AP/AC treatment. The heterogeneity of AP/AC protocols does not allow to establish the benefit of stopping those drugs before surgery in terms of avoiding blood transfusions when performing a VTUR.

Leiomyosarcoma of the inferior vena cava in a patient with double inferior vena cava.

BACKGROUND: Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor arising from its smooth muscle cells. METHODS AND RESULTS: We report the case of a 38-year-old woman presenting with back pain and weight loss who was diagnosed with a 22-cm leiomyosarcoma of the right IVC and thrombosis of the left IVC. The patient is alive and free of recurrence a year after radical tumor resection with removal of the affected IVC, reconstruction with polytetrafluoroethylene prosthetic graft, and anastomosis of both right and left IVC. CONCLUSIONS: Leiomyosarcoma is a rare and aggressive tumor with a deceitful course. Radical surgical en bloc resection is the mainstay of treatment for IVC leiomyosarcomas. For an adequate restoration of venous return, complex vascular repair may be necessary.

Rabdomiosarcoma paratesticular metastasico / Rhabdomyosarcoma metastatic paratesticular

OBJETIVOS: Presentar un caso de rabdomiosarcoma paratesticular metastásico en un varón de 14 años de edad, clasificado como estadio IIb según el IRSG (Intergroup Rhabdomyosarcoma Study Group).MÉTODOS: Tras recibir tratamiento mediante cirugía radical (orquiectomía más linfadenectomía retroperitoneal), poliquimioterapia y radioterapia, presentó buena respuesta inicialmente. RESULTADOS: A los 12 meses de la intervención quirúrgica el paciente se encuentra libre de enfermedad.CONCLUSIONES: Resultan fundamentales la quimioterapia y/o radioterapia adyuvantes para el tratamiento de estas neoplasias(AU)

OBJECTIVES: To report one case of metastatic paratesticular rhabdomyosarcoma in a 14 years old patient, classified as stage IIb (IRSG).METHODS: After treatment with radical surgery (orchiectomy and lymphadenectomy), polychemotherapy and radiotherapy, showed good evolution initially.RESULTS: 12 months after surgery the patient is disease free.CONCLUSIONS: Adjuvant treatment is very important in the prognosis of this kind of tumors(AU)

[Histological characteristics of head and neck paragangliomas]. / Anatomía patológica de los paragangliomas cervicocefálicos.

Paragangliomas arise from the extra-adrenal paraganglion system. Histologically, paragangliomas are usually easy to diagnose, with well-defined characteristics. These lesions are clearly delimited and highly vascular and are composed of cell balls (Zellballen) separated by thin fibrous septa. These cell balls are composed of two types of cells: chief cells and sustentacular cells. Other, less frequent patterns, which are nearly always focal, can also be found and hamper diagnosis: angiomatoid, fusocellular and clear cell. Some paragangliomas show intense fibrosis, which can compress and distort the cell balls, giving rise to a pseudoinfiltrative appearance (sclerosing paragangliomas). With immunohistochemical techniques, the chief cells are positive for neuroendocrine markers (neuron specific enolase, chromogranin A, synaptophysin, serotonin) while sustentacular cells are positive for S-100 protein. Ultrastructurally, the chief cells contain neurosecretory granules with dense centers and simple intercellular junctions without desmosomes. From a practical point of view, paragangliomas can be divided into three groups: non-invasive (circumscribed or encapsulated), locally invasive and metastatic. Although some invasive tumors can be fatal, there are no histological data that can predict the malignancy of paragangliomas, and the only absolute criterion for malignancy is the presence of metastasis.

Anatomía patológica de los paragangliomas cervicocefálicos / Histological characteristics of head and neck paragangliomas

Los paragangliomas son tumores que derivan del sistema paragangliónico extra adrenal. Desde el punto de vista histológico, los paragangliomas son, por lo general, tumores de fácil diagnóstico, con unas características bien definidas. Son lesiones bien delimitadas, muy vascularizadas y formadas por nidos celulares (Zellballen) separados por finos septos conjuntivos. Los nidos celulares están constituidos por 2 tipos de células: las principales y las sustentaculares. En ocasiones, existen otros patrones, menos frecuentes y casi siempre focales, que pueden dificultar el diagnóstico: angiomatoide, fusocelular y de células claras. Algunos paragangliomas se acompañan de intensa fibrosis, que puede comprimir y distorsionar los nidos celulares, dando una apariencia seudoinfiltrativa (paragangliomas esclerosantes). Con técnicas inmunohistoquímicas, las células principales son positivas para marcadores neuroendocrinos (enolasaneuronal específica, cromogranina A, sinaptofisina, serotonina) y las células sustentaculares para proteína S-100. Desde el punto de vista ultra estructural, las células principales muestran gránulos de centro denso, de tipo neurosecretor y uniones simples intercelulares sin desmosomas. Desde un punto de vista práctico, los paragangliomas pueden dividirse en 3 grupos: no invasivos (circunscritos o encapsulados), localmente invasivos y metastásicos. A pesar de que algunos tumores de crecimiento infiltrante pueden ser letales, no existe ningún dato histopatológico que pueda predecir el comportamiento maligno de los paragangliomas, y sólo la existencia de metástasis es criterio absoluto de malignidad (AU)

Paragangliomas arise from the extra-adrenal paraganglion system. Histologically, paragangliomas are usually easy to diagnose, with well-defined characteristics. These lesions are clearly delimited and highly vascular and are composed of cell balls (Zellballen) separated by thin fibrous septa. These cell balls are composed of two types of cells: chief cells and sustentacular cells. Other, less frequent patterns, which are nearly always focal, can also be found and hamper diagnosis: angiomatoid, fusocellular and clear cell. Some paragangliomas show intense fibrosis, which can compress and distort the cell balls, giving rise to a pseudo -infiltrative appearance (sclerosing paragangliomas). Withimmunohistochemical techniques, the chief cells are positive for neuroendocrine markers (neuron specific enolase, chromogran in A, synaptophys in, serotonin) while sustentacular cells are positive for S-100 protein. Ultrastructurally, the chief cells contain neurosecretory granules with densecenters and simple intercellular junctions without desmosomes. From a practical point of view, paragangliomas can be divided into three groups: non-invasive (circumscribed or encapsulated), locally invasive and metastatic. Although some invasive tumors can be fatal, there are no histological data that can predict the malignancy of paragangliomas, and the only absolute criterion for malignancy is the presence of metast (AU)

Hemorragia gastrointestinal recurrente por hemangioma mesentérico con afección del intestino delgado / Recurrent gastrointestinal bleeding or mesenteric hemangioma with small intestine involvement

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Leucemia cutánea aleucémica: presentación de un caso / Aleukemic leukemia cutis: a report of one case

Se denomina leucemia cutánea aleucémica a la invasión cutánea por células leucémicas en ausencia de afectación de sangre periférica y/o médula ósea. Aunque se desconocen los mecanismos patogénicos de esta enfermedad, está ampliamente aceptado su pronóstico grave. En la mayoría de los casos publicados se describe la expresión completa del proceso hematológico (con mayor frecuencia leucemias agudas monocíticas o granulocíticas) en los 10-14 meses posteriores al inicio del cuadro cutáneo, así como una supervivencia media de unos 22 meses. Presentamos el caso de un paciente varón de 77 años con leucemia cutánea aleucémica que inició la enfermedad en forma de múltiples lesiones papulonodulares eritematovioláceas en tronco y cabeza, con ausencia de afectación de sangre periférica y alteraciones incipientes en médula ósea. El estudio inmunohistoquímico, tanto de las lesiones cutáneas como de médula ósea, demostró la invasión de ambos tejidos por células tumorales pertenecientes a la estirpe monocitohistiocitaria. El paciente presentó una desaparición espontánea de las lesiones cutáneas, así como una ausencia de progresión del cuadro hematológico en los 9 meses posteriores al diagnóstico, a pesar de no haberse sometido a ningún tratamiento. Destacamos la baja incidencia de asociación entre síndromes mielodisplásicos y leucemias cutáneas aleucémicas, así como la sorprendente resolución espontánea de las lesiones cutáneas en nuestro caso (AU)