Surgical and postsurgical management of abdominal paragangliomas and pheochromocytomas.

PURPOSE: To describe our current protocol for surgical and postsurgical management of abdominal paragangliomas (PGLs) and pheochromocytomas, with a special focus on multidisciplinary management in centres with experience. METHODS: The physicians involved in the management of patients with abdominal PGLs and pheochromocytomas of our hospital reviewed systematically current knowledge on the surgical management of abdominal PGLs and pheochromocytomas. RESULTS: Currently, surgery is considered the treatment of choice for abdominal PGLs and pheochromocytomas. The choice of surgical approach is determined based on the location of the lesion, size, patient́s body habitus and the likelihood of malignancy. Laparoscopic surgery is usually considered the gold standard approach for pheochromocytomas, but open access should be considered in invasive and/or potentially malignant tumours >8-10 cm and for abdominal PGLs. Postsurgical management of pheochromocytomas and PGLs includes close hemodynamic monitoring and treatment of postsurgical complications, the pathological study of the surgical specimen, reassessment of hormonal and/or radiological status and planning of follow-up based on the risk of recurrence and malignancy. CONCLUSION: Surgery represents the treatment of choice of most abdominal PGLs and pheochromocytomas. Optimal postsurgical evaluation, including hemodynamic, pathological, hormonal, and radiological evaluation, should be performed by a multidisciplinary team specializing in PGL/pheochromocytoma management.

Manejo quirúrgico y posquirúrgico de paragangliomas abdominales y feocromocitomas / Surgical and postsurgical management of abdominal paragangliomas and pheochromocytomas

Objetivo Describir nuestro protocolo actual para el manejo quirúrgico y posquirúrgico de los paragangliomas abdominales (PGL) y los feocromocitomas, con especial atención en el manejo multidisciplinar en centros con experiencia. Métodos Los facultativos implicados en el tratamiento de pacientes con PGL abdominales y feocromocitomas de nuestro hospital revisaron sistemáticamente la evidencia científica actualmente disponible sobre el tratamiento quirúrgico de los PGL abdominales y feocromocitomas. Resultados La cirugía se considera el tratamiento de primera elección para los PGL abdominales y feocromocitomas. La decisión sobre el tipo de abordaje quirúrgico se basa en la localización y el tamaño de la lesión, la constitución corporal del paciente y la probabilidad estimada de malignidad. La cirugía laparoscópica suele considerarse el abordaje de referencia para los feocromocitomas, pero en los tumores invasivos y/o potencialmente malignos de más de 8-10 cm y en los PGL abdominales debe considerarse el abordaje abierto. El tratamiento posquirúrgico de los feocromocitomas y los PGL incluye una monitorización hemodinámica estrecha, el tratamiento de las complicaciones posoperatorias, el estudio patológico de la muestra quirúrgica, la reevaluación del estado hormonal y/o radiológico y la planificación del seguimiento en función del riesgo de recurrencia y malignidad. Conclusión La cirugía representa el tratamiento de elección de la mayoría de los PGL abdominales y feocromocitomas. La evaluación posoperatoria óptima, que incluye la evaluación hemodinámica, patológica, hormonal y radiológica, debe ser realizada por un equipo multidisciplinar especializado en el tratamiento de PGL/feocromocitomas (AU)

Purpose To describe our current protocol for surgical and postsurgical management of abdominal paragangliomas (PGLs) and pheochromocytomas, with a special focus on multidisciplinary management in centres with experience. Methods The physicians involved in the management of patients with abdominal PGLs and pheochromocytomas of our hospital reviewed systematically current knowledge on the surgical management of abdominal PGLs and pheochromocytomas. Results Currently, surgery is considered the treatment of choice for abdominal PGLs and pheochromocytomas. The choice of surgical approach is determined based on the location of the lesion, size, patient́s body habitus and the likelihood of malignancy. Laparoscopic surgery is usually considered the gold standard approach for pheochromocytomas, but open access should be considered in invasive and/or potentially malignant tumours > 8-10 cm and for abdominal PGLs. Postsurgical management of pheochromocytomas and PGLs includes close hemodynamic monitoring and treatment of postsurgical complications, the pathological study of the surgical specimen, reassessment of hormonal and/or radiological status and planning of follow-up based on the risk of recurrence and malignancy. Conclusion Surgery represents the treatment of choice of most abdominal PGLs and pheochromocytomas. Optimal postsurgical evaluation, including hemodynamic, pathological, hormonal, and radiological evaluation, should be performed by a multidisciplinary team specializing in PGL/pheochromocytoma management (AU)

Tumour size in adrenal tumours: its importance in the indication of adrenalectomy and in surgical outcomes-a single-centre experience.

OBJECTIVE: To evaluate the relevance of tumour size in adrenal tumours in the estimation of malignancy risk and in the outcomes of adrenalectomy. METHODS: We evaluate the histological results and surgical outcomes (intraoperative and postsurgical complications) in a retrospective single-centre cohort of patients without history of active extraadrenal malignancy with adrenal tumours consecutively operated in our centre during January 2010 and December 2020. We compared these results in lesions smaller and larger than 40, 50, and 60 mm. RESULTS: Of 131 patients with adrenal tumours who underwent adrenalectomy, 76 (58.0%) had adrenal masses measuring ≥ 40 mm; 47 were > 50 mm and 28 > 60 mm. The final diagnosis was adrenocortical carcinoma (ACC) in 7 patients, pheochromocytoma in 35, and benign lesions in the remaining. All patients with ACC had adrenal masses > 50 mm, with Hounsfield units > 40 and low lipidic content in the CT. The risk of ACC and pheochromocytoma increased as tumour size did. The diagnostic accuracy of tumour size was quite good for the prediction of ACC (AUC-ROC 0.883). Nevertheless, when only adrenal tumours with HU < 40 were considered, the risk of ACC was 0% independent of tumour size. For pheochromocytomas, the risk was of 8.6% independent of tumour size for lesions with < 20HU. The risk of intraoperative and postoperative complications was independent of tumour size. CONCLUSION: Risk of malignancy and of pheochromocytoma increased as tumour size increased, but, in the presurgical estimation of malignancy risk and of pheochromocytoma, not only tumour size, also lipidic content and other radiological features, should be considered. The risk of complications was independent of tumour size, but hospital stay was longer in patients with complication or open approach.

Protocol for presurgical and anesthetic management of pheochromocytomas and sympathetic paragangliomas: a multidisciplinary approach.

OBJECTIVE: To offer a practical guide for the presurgical and anesthetic management of pheochromocytomas and sympathetic paragangliomas (PGLs). METHODS: This protocol was based on a comprehensive review of the literature and on our own multidisciplinary team's experience from managing pheochromocytoma and sympathetic PGLs at a referral center. RESULTS: Patients with pheochromocytomas and sympathetic paragangliomas (PGLs) may develop potentially life-threatening complications, especially during surgical procedures. A complete biochemical, radiological, genetic, and cardiological assessment is recommended in the preoperative stage as it provides an evaluation of the risk of surgical complications and malignancy, allowing individualization of the presurgical treatment. Treatment with α-blockade and proper volume expansion in the preoperative stage significantly reduces the perioperative morbidity. During surgery, the anesthesiologist should look for a deep anesthetic level that inhibits the cardiovascular effects of catecholamines to minimize the risk of intraoperative complications. CONCLUSIONS: An optimal presurgical evaluation of pheochromocytomas/ sympathetic PGL requires a multidisciplinary approach, including a complete hormonal, radiological, cardiac, genetic, and functioning evaluation in most cases. A proper preoperative evaluation in combination with strict blood pressure and heart rate control, and blood volume status optimization, will significantly reduce the risk of intraoperative and perioperative complications. In those patients who unfortunately develop intraoperative complications, the role of the anesthesiologist is essential since the selection of the appropriate management has a direct impact on morbimortality reduction.

Pheochromocytoma: A three-decade clinical experience in a multicenter study.

OBJECTIVE: To analyze the clinical and analytical features, diagnostic tests, therapies, and outcomes of pheochromocytoma (PCC). DESIGN AND METHODS: A multicenter retrospective study in surgically treated patients with PCC followed in 3 Spanish tertiary referral hospitals. RESULTS: A total of 106 patients (61 [57.5%] women, mean age 52.3 ±â€¯14.8 years) were evaluated. At diagnosis, PCC was symptomatic in 62% and sporadic in 83%. Patients with familial PCC were significantly younger than those with sporadic disease (40.8 ±â€¯14.2 years vs 54.5 ±â€¯13.9 years, p < .001). Familial PCCs were more frequently associated with MEN2A (n = 8). Levels of 24-h urinary fractionated metanephrines were positively related to tumor size. The maximum tumor diameter was 4.3 cm (3-6 cm); 27.7% of the patients had tumors ≥6 cm. Incidental PCCs were significantly smaller than symptomatic PCCs (3.4 cm [2.4-5.0 cm] vs 5.6 cm [4.0-7.0 cm], p < .001). Scintigraphy by ¹²³I-metaiodobenzylguanidine showed a high sensitivity (81.9%). Preoperative alpha blockade with phenoxybenzamine was used in 93.6% and doxazosin in the rest. Laparoscopic surgery was used in 2/3 of the patients, with a low conversion (1.9%) to open surgery. Perioperative complications appeared in approximately 20% of patients, mainly hypertensive crisis (9.4%). Recurrent disease appeared in 10%, and malignant PCC was uncommon (6.3%). CONCLUSIONS: PCCs surgically treated in Spain are usually large, symptomatic, and sporadic tumors diagnosed around the sixth decade of life. Hereditary PCC is usually associated with MEN2A. The main type of surgical technique used is laparoscopic surgery, and the prevalence of metastatic PCC is low.

Feocromocitoma: experiencia clínica de tres décadas en un estudio multicéntrico / Pheochromocytoma: A three-decade clinical experience in a multicenter study

Objetivo Analizar las características clínicas y analíticas, las pruebas diagnósticas, los tratamientos y los resultados del feocromocitoma (FCC). Diseño y métodos Estudio multicéntrico retrospectivo en pacientes con FCC tratados quirúrgicamente y seguidos en 3 hospitales terciarios de referencia españoles. Resultados Se analizó a un total de 106 pacientes (61 [57,5%] mujeres, edad media 52,3 ± 14,8 años). En el diagnóstico, el FCC fue sintomático en el 62% de los casos y esporádico en el 83%. Los pacientes con FCC familiar eran significativamente más jóvenes que aquellos que presentaban la enfermedad esporádica (40,8 ± 14,2 años vs. 54,5 ± 13,9 años, p < 0,001). El FCC familiar se asociaba con mayor frecuencia a la MEN2A (n = 8). Los niveles de metanefrinas en orina de 24h se relacionaron positivamente con el tamaño del tumor. El diámetro tumoral máximo fue 4,3cm (3-6cm); el 27,8% de los pacientes tenían tumores ≥ 6cm. Los FCC incidentales eran significativamente más pequeños que los FCC sintomáticos (3,4cm [2,4-5,0cm] vs. 5,6cm [4,0-7,0cm], p < 0,001). La gammagrafía con 123I-metaiodobencilguanidina mostró una alta sensibilidad (81,9%). En el 93,6% de los casos se usó el bloqueo alfa preoperatorio con fenoxibenzamina y con doxazosina en los demás casos. En 2/3 de los pacientes se empleó la cirugía laparoscópica, con una baja tasa de conversión (1,9%) a cirugía abierta. Aproximadamente en el 20% de los pacientes aparecieron complicaciones perioperatorias, principalmente crisis hipertensivas (9,4%). La enfermedad recurrente apareció en el 10% de los casos y el FCC maligno fue raro (6,3%). Conclusiones Los FCC tratados quirúrgicamente en España suelen ser tumores grandes, sintomáticos y esporádicos diagnosticados alrededor de la sexta década de vida. El FCC hereditario está generalmente asociado con MEN2A. La cirugía laparoscópica es el tipo principal de técnica quirúrgica utilizada y la prevalencia del FCC metastásico es baja (AU)

Objective To analyze the clinical and analytical features, diagnostic tests, therapies, and outcomes of pheochromocytoma (PCC). Design and methods A multicenter retrospective study in surgically treated patients with PCC followed in 3 Spanish tertiary referral hospitals. Results A total of 106 patients (61 [57.5%] women, mean age 52.3 ± 14.8 years) were evaluated. At diagnosis, PCC was symptomatic in 62% and sporadic in 83%. Patients with familial PCC were significantly younger than those with sporadic disease (40.8 ± 14.2 years vs. 54.5 ± 13.9 years, p<.001). Familial PCCs were more frequently associated with MEN2A (n=8). Levels of 24-h urinary fractionated metanephrines were positively related to tumor size. The maximum tumor diameter was 4.3cm (3-6cm); 27.7% of the patients had tumors ≥6cm. Incidental PCCs were significantly smaller than symptomatic PCCs (3.4cm [2.4-5.0cm] vs. 5.6cm [4.0-7.0cm], p<.001). Scintigraphy by 123I-metaiodobenzylguanidine showed a high sensitivity (81.9%). Preoperative alpha blockade with phenoxybenzamine was used in 93.6% and doxazosin in the rest. Laparoscopic surgery was used in 2/3 of the patients, with a low conversion (1.9%) to open surgery. Perioperative complications appeared in approximately 20% of patients, mainly hypertensive crisis (9.4%). Recurrent disease appeared in 10%, and malignant PCC was uncommon (6.3%). Conclusion PCCs surgically treated in Spain are usually large, symptomatic, and sporadic tumors diagnosed around the sixth decade of life. Hereditary PCC is usually associated with MEN2A. The main type of surgical technique used is laparoscopic surgery, and the prevalence of metastatic PCC is low (AU)

Mononeuritis múltiple vasculítica asociada a vasculopatía livedoide / Vasculitic mononeuritis multiplex associated with livedoid vasculopathy

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Pheochromocytoma: A three-decade clinical experience in a multicenter study. / Feocromocitoma: experiencia clínica de tres décadas en un estudio multicéntrico.

OBJECTIVE: To analyze the clinical and analytical features, diagnostic tests, therapies, and outcomes of pheochromocytoma (PCC). DESIGN AND METHODS: A multicenter retrospective study in surgically treated patients with PCC followed in 3 Spanish tertiary referral hospitals. RESULTS: A total of 106 patients (61 [57.5%] women, mean age 52.3 ± 14.8 years) were evaluated. At diagnosis, PCC was symptomatic in 62% and sporadic in 83%. Patients with familial PCC were significantly younger than those with sporadic disease (40.8 ± 14.2 years vs. 54.5 ± 13.9 years, p<.001). Familial PCCs were more frequently associated with MEN2A (n=8). Levels of 24-h urinary fractionated metanephrines were positively related to tumor size. The maximum tumor diameter was 4.3cm (3-6cm); 27.7% of the patients had tumors ≥6cm. Incidental PCCs were significantly smaller than symptomatic PCCs (3.4cm [2.4-5.0cm] vs. 5.6cm [4.0-7.0cm], p<.001). Scintigraphy by 123I-metaiodobenzylguanidine showed a high sensitivity (81.9%). Preoperative alpha blockade with phenoxybenzamine was used in 93.6% and doxazosin in the rest. Laparoscopic surgery was used in 2/3 of the patients, with a low conversion (1.9%) to open surgery. Perioperative complications appeared in approximately 20% of patients, mainly hypertensive crisis (9.4%). Recurrent disease appeared in 10%, and malignant PCC was uncommon (6.3%). CONCLUSIONS: PCCs surgically treated in Spain are usually large, symptomatic, and sporadic tumors diagnosed around the sixth decade of life. Hereditary PCC is usually associated with MEN2A. The main type of surgical technique used is laparoscopic surgery, and the prevalence of metastatic PCC is low.

Postoperative management of patients with pituitary tumors submitted to pituitary surgery. Experience of a Spanish Pituitary Tumor Center of Excellence.

There is a lack of evidence on timing, frequency, and duration of postoperative endocrine, radiologic, and ophthalmologic assessments that should be performed after pituitary surgery (PS). However, it is known that careful optimization of treatment and follow-up strategies as well as a multidisciplinary approach may have a significant impact on long-term outcomes, improving surgical results, minimize complications and facilitate their correct treatment if occurring, and optimize the hormonal, ophthalmological, and radiological reassessment throughout the follow-up. Considering that there are no specific guidelines on the postoperative management of patients with pituitary tumors (PT), we present our protocol for the postoperative management of patients with PT. It has been elaborated by the multidisciplinary team of a Spanish Pituitary Tumor Center of Excellence (PTCE) that includes at least one neurosurgeon, ENT, neuroradiologist, neuro-ophthalmologist, endocrine pathologist and endocrinologist specialized in pituitary diseases. We elaborated this guideline with the aim of sharing our experience with other centers involved in the management of PT thereby facilitating the postoperative management of patients submitted to PS.

Angiolipomas espinales epidurales: presentación de 7 casos y revisión de la literatura / Spinal extradural angiolipomas: 7 new cases and review of the literatura

Introducción: Presentamos nuestra experiencia en el manejo diagnóstico y terapéutico de los angiolipomas espinales. Métodos: Hemos realizado una revisión en nuestra base de datos quirúrgica correspondiente al diagnóstico patológico de angiolipoma, recogiendo las variables epidemiológicas, clínicas, diagnósticas, terapéuticas y de seguimiento de cada caso. Para la valoración funcional de los pacientes se utilizó la escala de mielopatía modificada de la Asociación Japonesa de Ortopedia (EAJO). Resultados: Obtuvimos un total de 7 casos, con edades comprendidas entre los 42 y 63 años; 4 eran mujeres. Todas las lesiones se localizaron en el espacio epidural torácico, con extensión extrarraquídea en 2 casos. La presentación más frecuente fue la combinación de dolor y mielopatía progresiva, con una puntuación entre 5 y 9 en la EAJO. Los hallazgos radiológicos fueron variables, claramente influenciados por la proporción de los componentes tumorales, identificándose un caso de variante celular, uno de predominio angiomatoso y otro mixoide. Cuatro estudios evidenciaban signos de afectación ósea. Tras el tratamiento quirúrgico se registraron puntuaciones óptimas en la EAJO, salvo en un caso (puntuación de 10 en la EAJO), sin evidenciarse recurrencias tras un periodo de seguimiento que varió entre uno y 5 años. Conclusiones: Los angiolipomas epidurales pueden dar lugar a manifestaciones clínicas y radiológicas muy variables. Su tratamiento quirúrgico debe diseñarse de acuerdo a la extensión de la tumoración, incluyendo en ocasiones un procedimiento reconstructivo espinal, y se traduce en resultados excelentes con independencia de la situación clínica previa. La posibilidad de recurrencia es baja, incluso en caso de resección incompleta

Introduction: We present our experience with the diagnosis and treatment of spinal angiolipomas (SAL). Methods: We used our database of surgical patients to gather epidemiological, clinical, diagnostic, treatment, and follow-up data from confirmed cases of SAL. Patients’ functional status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score for thoracic myelopathy. Results: We included 7 patients with ages ranging between 42 and 63 years; 4 were women. Lesions in all patients were located in the thoracic epidural space; in 2 cases, lesions extended outside the spinal canal. The most frequent form of presentation was pain combined with progressive myelopathy; patients scored 5 to 9 points on the mJOA scale. Radiological findings varied; this variability clearly depended on the proportion of tumour components (cellular variant in one case, predominantly angiomatous in another, and myxoid in yet another case). Four cases displayed radiological signs of bone involvement. Surgery resulted in excellent mJOA scores except in one case (mJOA score of 10); no recurrences were detected during follow-up (periods ranging from 1 to 5 years). Conclusions: Extradural SAL can present with highly variable radiological and clinical features. Surgery must be tailored to each individual situation according to tumour extension; in some cases, it may include spinal reconstruction. Surgery results in excellent outcomes regardless of preoperative functional status and it is associated with low recurrence rates, even in case of incomplete resection

Clinical features, diagnosis and therapy of pituicytoma: an update.

BACKGROUND: Pituicytomas (PTs) are extremely rare, low-grade glial tumors closely related to the neurohypophyseal axis. Definite conclusions concerning the optimal diagnostic and therapeutic approach to these neoplasms are lacking to date, as most of this information has been presented as case reports. METHODS: Retrospective review of case reports published in the scientific literature to date, including a new illustrative example treated in our department. RESULTS: 116 cases were collected. PTs had a higher prevalence in the fifth and sixth decades of life, with a slight male predominance. Main symptoms, which tended to be progressive, included visual field defects and pituitary-hypothalamic dysfunction. Radiologically, PTs were found anywhere along the hypothalamic-pituitary axis mimicking other, more frequent tumors growing in this anatomical region. Surgical treatment included both transcranial or transsphenoidal approaches, and resulted in gross total resection and morbidity rates of 46.8 and 59%, respectively; the latter essentially consisted in anterior and posterior pituitary dysfunction, with limited impact on daily quality of life. CONCLUSIONS: Due to both low frequency and the absence of pathognomonic clinical and/or radiological features, formulating a suspicion diagnosis of PT represents a considerable challenge even for experienced professionals. The indication for treatment should be made on an individual basis, but it is inescapable in the presence of a visual field defect. The surgical approach has to be tailored according to the topography of the tumor and preoperative symptoms; the greatest challenges in accomplishing a gross total removal are represented by the degree of adherence and vascularization of the PT.

Spinal extradural angiolipomas: 7 new cases and review of the literature. / Angiolipomas espinales epidurales: presentación de 7 casos y revisión de la literatura.

INTRODUCTION: We present our experience with the diagnosis and treatment of spinal angiolipomas (SAL). METHODS: We used our database of surgical patients to gather epidemiological, clinical, diagnostic, treatment, and follow-up data from confirmed cases of SAL. Patients' functional status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score for thoracic myelopathy. RESULTS: We included 7 patients with ages ranging between 42 and 63 years; 4 were women. Lesions in all patients were located in the thoracic epidural space; in 2 cases, lesions extended outside the spinal canal. The most frequent form of presentation was pain combined with progressive myelopathy; patients scored 5 to 9 points on the mJOA scale. Radiological findings varied; this variability clearly depended on the proportion of tumour components (cellular variant in one case, predominantly angiomatous in another, and myxoid in yet another case). Four cases displayed radiological signs of bone involvement. Surgery resulted in excellent mJOA scores except in one case (mJOA score of 10); no recurrences were detected during follow-up (periods ranging from 1 to 5 years). CONCLUSIONS: Extradural SAL can present with highly variable radiological and clinical features. Surgery must be tailored to each individual situation according to tumour extension; in some cases, it may include spinal reconstruction. Surgery results in excellent outcomes regardless of preoperative functional status and it is associated with low recurrence rates, even in case of incomplete resection.

Colonic perforation secondary to metastatic lung adenocarcinoma during anti-TNF treatment for ankylosing spondylitis.

Biological agents targeting inflammatory cytokines such as tumour necrosis factor alpha (TNF-α) have emerged in recent years as effective medications for a variety of inflammatory arthropathies. Although the relationship between the use of anti-TNF drugs and an increase in the rate of infections is well established, the role of these drugs in the development of different types of cancer is unclear. Randomized clinical trials and national registries have not demonstrated a significant increase in the risk of cancer in patients treated with anti-TNF drugs, but numerous cases of the appearance of malignant tumors in patients receiving these drugs have been reported. We describe the case of a 73-year-old man, ex-smoker, who developed a lung cancer during treatment with infliximab further complicated by perforation of a metastasis in the sigmoid colon, which is a very infrequent event in the course of this malignancy. A few similar cases previously reported in the literature are reviewed.

Case report of splenic artery steal syndrome: demonstration of portal hyperflow mechanism by anatomic variant of the splenic artery and correlation with Doppler rates.

We report the case of a liver transplant recipient who developed a "splenic artery steal syndrome" (SASS) successfully treated by partial splenic embolization (PSE). Interestingly, because the patient presented an anatomic variant of the splenic artery (SA) originating from the superior mesenteric artery (SMA), improvement was observed in hepatic artery (HA) flow following PSE that could only be explained by decreased portal perfusion and not by the derivation from the SA.

Intralymphatic granulomas as a pathogenic factor in cheilitis granulomatosa/Melkersson-Rosenthal syndrome: report of a case with immunohistochemical and molecular studies.

Orofacial granulomatosis, an uncommon immunologically mediated disorder, includes cheilitis granulomatosa and Melkersson-Rosenthal syndrome. It is clinically characterized by recurrent or persistent swelling of the orofacial tissues with a spectrum of other orofacial features and sometimes with neurological symptoms. The pathological findings are varied but are often characterized by the presence of noncaseating granuloma. We present a new case of orofacial granulomatosis with unusual histopathological findings, namely, intralymphatic granulomas. These may be the cause of the tissue edema. We demonstrated, by immunohistochemical studies, the lymphatic nature of the vessels affected by the granulomatous process.

[GATA-2 gene expression in leukemia patients and its significance].

OBJECTIVE: To investigate GATA-2 gene expression in leukemia patients and its clinical significance. METHODS: GATA-2 gene transcripts were detected by reverse transcriptase polymerase chain reaction (RT-PCR), and bcr/abl or PML/RAR alpha transcripts were detected in patients with chronic myeloid leukemia (CML) and acute promyelocytic leukemia (APL), respectively. RESULTS: The GATA-2 transcripts were revealed in 93% of acute myeloid leukemia (AML), in 70% of acute lymphoblastic leukemia (ALL), and 83% of CML patients, but not in normal bone marrow and peripheral blood cells. GATA-2 expression in remission patients was similar to newly diagnosed or relapsed patients. In post-transplantation patients, GATA-2 expression was significantly decreased. In these cases GATA-2 transcripts could be detected with bcr/abl fusion gene disappeared in 2 of 12 CML, whereas neither GATA-2 nor PML/RARalpha transcripts could be detected in APL. CONCLUSION: GATA-2 transcripts was highly expressed in leukemia patients and had no change in remission but significantly decreased in post-transplantation patients. Detection of GATA-2 expression would give information about the residual leukemia stem cells.

[Cytogenetic analysis and bcr/abl mRNA detection in chronic myeloid leukemia].

OBJECTIVE: To define the relationship between Ph chromosome and BCR/ABL mRNA expression in chronic myeloid leukemia (CML) patients, and compare cytogenetic analysis and PCR method. METHODS: Marrow or peripheral blood samples from 33 CML patients were analyzed by R banding technique and PCR method. Eleven patients who received allogeneic BMT or alpha-IFN + low dose HU were followed up. RESULTS: In 33 patients, 29 were Ph(+), 30 expressed BCR/ABL mRNA. In 6 patients who received BMT, 4 were Ph(-), 2 were Ph(+). However, 2 patients were (-), 4 were (+) by PCR method. In 5 patients who received alpha-IFN, the percentage of Ph(+) cells reduced after 6 and 12 months treatment than before treatment in 2 cases respectively. In 41 samples, 34 cases were consistent by 2 methods. CONCLUSION: Each method, Cytogenetic analysis or PCR has its advantage and defect. The better method are integration of 2 methods in diagnosis and treatment monitoring of CML patients, so that provide real and beneficial information for clinic.