Miguel Echenique-Elizondo

Parathyroid Subcutaneous Pre-sternal Transplantation after Parathyroidectomy for Renal Hyperparathyroidism. Long-term Graft Function

BackgroundIn the setting of total parathyroidectomy (TPT) and parathyroid transplantation (PTx) for renal hyperparathyroidism (RHP), we evaluated long-term parathyroid graft function after subcutaneous pre-sternal transplantation (SCPTx). Because parathyroid glands are surrounded by fatty tissue, we postulated that results of subcutaneous implantation of parathyroid tissue after total parathyroidectomy for renal hyperparathyroidism could be at least as successful as intramuscular grafting, but without its complicationsPatients and MethodsThe study, a prospective open efficacy study of postoperative (po) diagnostic monitoring of intact parathyroid hormone (iPTH) on a cohort of surgical patients, was conducted within a university hospital with a dialysis unit. Thirty five patients (19 women and 16 men) operated on for renal hyperparathyroidism underwent TPT and SCPTx for RHP at the Department of General Surgery and the Department of Nephrology. Donostia Hospital. San Sebastián. Gipuzkoa. Spain, from January 2002 to December 2005. Follow-up ranges from 6 months to 42 months (median: 15.4 months). The main outcome measure was evaluation of graft function by measurement of iPTH plasma level, based on serum levels of iPTH before operation and 24 h and 1, 3, 5, 15, 30, 60, 100, and 150 weeks after surgeryResultsAverage preoperative iPTH values were 1,341.52 + 367.78 pg/ml (mean ± SD) (range: 493–2,180). After TPT and PSCTx, iPTH levels became undetectable in all patients at 24 h. A level of 50 pg/ml was established as the criterion of adequate parathyroid graft function. Values obtained at the various time intervals were as follows: 14.14 + 7.73 1 pg/ml (mean ± SD) (range: 6–36) after 1 week, 53 + 77.33 pg/ml (mean ± SD) (range: 35–74) after 5 weeks, 62.95 + 20.93 pg/ml (mean ± SD) (range: 11–89) after 15 weeks, 77.54 + 18.84 pg/ml (mean ± SD) (range: 24.6–104.2) after 30 weeks, 109.29 + 50.22 pg/ml (mean ± SD) (range: 54–327) after 60 weeks, 134.21 + 128.64 pg/ml (mean ± SD) (range: 43–712) after 100 weeks, and 122.84 + 117.54 pg/ml (mean ± SD) (range: 68–723) after 150 weeks. Prevalence of hypoparathyroidism (intact parathyroid hormone serum level < 20 pg/ml with a normal or low serum calcium concentration) was 2/35 (5.71%) by week 60 and recovered by week 100. Graft-related recurrence was 2.85% (1/35).ConclusionsSubcutaneous pre-sternal transplantation (SCPTx) after TPT and PTx for secondary (RHP) is an adequate method to replace muscular forearm parathyroid transplantation and avoid its complications. Functioning results of total parathyroidectomy and presternal subcutaneous grafting compare favorably with the published data on other surgical techniques proposed for the treatment of renal hyperparathyroidism. Results of long-term follow-up exceed previously reported results.

Liposarcoma retroperitoneal gigante

Liposarcoma is a malignancy of fat cells and is the most frequent soft tissue sarcoma localized in the retroperitoneum. It can reach substantial proportions. It is a slow-growing tumor, and the most frequent symptom is nonspecific abdominal pain and diffuse abdominal enlargement. Treatment is radical surgery and complete resection is essential for local control of the disease. We present a case of giant right retroperitoneal liposarcoma, which was well-encapsulated and could be completely excised. The patient is currently in follow up and at 2 years is disease-free.

Adenocarcinoma mucinoso en fístula perianal de largo tiempo de evolución tratado mediante QT-RT neoadyuvante y amputación abdominoperineal laparoscópica

Resumen Paciente varon con una fistula perianal de 30 anos de evolucion tratado en varias ocasiones. Presento un adenocarcinoma mucoide anal. Fue tratado con quimioterapia (5-FU y leucovorin) y radioterapia externa de forma preoperatoria y amputacion abdominoperineal laparoscopica asistida. El adenocarcinoma mucoide sobre fistula perianal cronica es un proceso infrecuente y de diagnostico tardio asociado a un mal pronostico.

Síndrome de Stewart-Treves

Lymphangiosarcoma is an uncommon vascular tumor that usually develops in longstanding lymphedema. We gathered the cases of lymphangioma observed in a hospital and attempted to analyze their characteristics: age, sex, localization, treatment and follow-up data. We studied five cases: three cases of Stewart-Treves syndrome after mastectomy and radiotherapy and two cases that developed in patients with late-onset congenital lymphedema. There were four women and one man. Radical surgery was performed in four patients. The techniques employed were: above-knee amputation (one patient), hip disarticulation (one patient), scapulo-humeral disarticulation (two patients) and gemcitabine and radiotherapy in one patient with metastatic disease at diagnosis. Three patients died in the first 14 months of follow-up, while two are disease free after 46 and 86 months respectively. This study confirms the poor prognosis of patients with Steward-Treves syndrome.

Squamous-cell carcinoma on long-lasting lymphoedema

A 63-year-old white male living in a rural area, with previously untreated congenital lymphoedema, was admitted to our clinic (figure 1). A histological analysis of a biopsy from the lesion showed squamous-cell carcinoma cells. Metastases were also found in the ulcerated inguinal-lymphnodes. The patient underwent lymphoedema-reducing surgery, with wide excision of the ulcerated area and inguinal lymphadenectomy. Unfortunately, he died of a massive pulmonary embolism 4 days after surgery even though adequate pharmacological and physical measures were taken. Lymphoedema 1 may be a primary or secondary occurrence following either disease or surgery. Primary lymphoedema is sometimes congenital (Milroy’s disease) but it may also occur at any time of life, particularly puberty. Secondary lymphoedema is the most common form however, and often the result of filariasis, which is particularly common in southeast Asia and Africa. In developed countries, postsurgical lymphoedema of the extremity is common, and complications of chronic limb-lymphoedema include recurrent cellulitis and lymphangiosarcoma. Although lymphangiosarcoma 2 is a recognised compli

Extended hemipelvectomy—quality of life 20 years later

A 20-year-old woman admitted to our clinic presented with a malignant osteoclastoma of the pelvis. She had been treated previously with couretage followed by 100 Gy of radiation therapy (figure 1). At that time, we did a radical extended hemipelvectomy (high amputation of the leg including the sacroiliac joint), histerectomy, right anexectomy, cistectomy, and rectal urinary diversion. The postoperative period was uneventful and no additional therapy was required. After 20 years, the patient is free of disease, has a satisfactory job, drives her own car, and is happy with her quality of life. In addition, she has not experienced phantom limb syndrome (figure 2). Early descriptions of hemipelvectomy emphasise the need for careful selection of patients and the importance of immediate replacement of blood loss. Current technical descriptions of this procedure have been published. 1 A recent report on a series of patients who underwent hemipelvectomy showed that this procedure has low mortality and acceptable survival. 2 Quality-of-life studies suggest that long-term morbidity is no greater than other cancer treatments. 3

Enfermedad de Castleman de localización retroperitoneal

La enfermedad de Castleman es un proceso linfoproliferativo no neoplásico poco frecuente y de etiología desconocida que se caracteriza por un importante crecimiento de los ganglios linfáticos. Puede ocurrir en cualquier cadena linfática del organismo, pero ocurre con mayor frecuencia (70%) en el mediastino1. Presentamos el caso de una mujer de 45 años de edad sin antecedentes clínicos de interés que consulta por presentar una masa en el abdomen. El examen físico es normal. No se aprecian adenomagalias. La exploración abdominal revela la existencia de una tumoración ovoidea en el hipocondrio izquierdo, de superficie lisa, indolora y móvil. La analítica es normal. La ecografía abdominal y la tomografía computarizada (TC) abdominopelviana informan de la existencia de un tumor de dimensiones 8,5 × 6,5 cm (fig. 1). No se identifican adenopatías en retroperitoneo ni en el mesenterio. Los estudios complementarios (determinación del valor de hormona luteínizante [LH], hormona foliculostimulante [FSH], 17β estradiol y cortisol basal, y tras estimulo con dexametasona) resultan normales, lo mismo que los valores de noradrenalina, adrenalina y dopamina en sangre y orina de 24 h. Se practica su resección quirúrgica. El estudio anatomopatológico corresponde a una angiofolicular o enfermedad de Castleman en su variante hialino-vascular. La evolución de la paciente en el postoperatorio fue satisfactoria. Se conocen 2 variantes histológicas de la enfermedad de Castleman: la hialino-vascular y la de predominio plasmocitario2. Generalmente, la primera suele ser asintomática mientras que la segunda se asocia a manifestaciones sistémicas en forma de múltiples adenomegalias, fiebre, infecciones recurrentes y alteraciones inmunológicas3,4, tales como anemia hemolítica, trombocitopenia, artritis reumatoide, lupus eritematoso sistémico, pérdida de peso e hipergammaglobulinemia policlonal, que constituyen generalmente las formas de enfermedad de Castleman multicéntrica2. Ésta ocurre con mayor frecuencia en pacientes ancianos3, y existe el riesgo de desarrollo de una enfermedad maligna, frecuentemente el sarcoma de Kaposi, los linfomas y la leucemia crónica. El tratamiento, en estos casos, suele ser eficaz con los corticoides o los regímenes quimioterápicos similares a los utilizados en casos de linfoma. El estudio anatomopatológico resulta del tipo hialino-vascular en un 90% en estos casos. La localización retroperitoneal es de aproximadamente el 3%. La ultrasonografía, la tomografía computarizada y la resonancia magnética han demostrado ser útiles en el diagnóstico de los procesos expansivos retroperitoneales. No obstante, la enfermedad de Castleman no es claramente distinguible de otros procesos solamente mediante técnicas de imagen ni en el curso de la intervención quirúrgica, y el estudio histopatológico es la clave del diagnóstico final correcto. La extirpación quirúrgica es el tratamiento de elección para la enfermedad localizada5. En casos de enfermedad multicéntrica, el tratamiento no está establecido. En estos casos, el tratamiento sistémico con esteroides o quimioterapia, o ambos combinados, ha ofrecido resultados variables.

Glucagonoma and necrolytic migratory erythema

54-year-old male with a history of necrolytic migratory erythema (NME) (Figs. 1 and 2) and glossitis. Routine blood tests were normal except for glucose: 145 mg/dL. Baseline plasma glucagon levels were 1200 pg/mL. Serum zinc was 97 mcg/dL. An abdominal CT scan showed a large mass involving the body and tail of the pancreas. A dis-tal pancreatectomy with splenectomy was performed and no evidence of metastatic disease was observed. The skin rash cleared within a week after the operation and the patient remains free of disease at 38 months following surgery. COMMENTS Glucagonoma syndrome is a paraneoplastic phenomenon comprising a pancreatic glucagon-secreting insular tumor, necrolytic migratory erythema (NME), diabetes, weight loss, anemia, stomatitis, thrombo-embolism, dyspepsia, and neu-ropsychiatric disturbances. The occurrence of one or more of these symptoms associated with a proven pancreatic neo-plasm fits this diagnosis (1). Other skin and mucosal changes such as atrophic glossitis, cheylitis, and inflammation of the oral mucosa may be found (2). Hyperglycemia may be included –multiple endocrine neoplasia syndrome, i.e., Zollinger-Ellison syndrome– or the disease may result from a glucagon-secreting tumor alone. These tumors are of slow growth and present with non-specific symptoms in early stages. At least 50% are metasta-tic at the time of diagnosis, and therefore have a poor prognosis. Fig. 2.-NME. Necrolytic migratory erythema. ENM. Eritema necrolítico migratorio.

The pattern of the descent of PTH measured by intraoperative monitoring of intact-PTH in surgery for renal hyperparathyroidism

BackgroundIn the setting of total parathyroidectomy and autotransplantation surgery (TPTxAS) treatment for secondary hyperparathyroidism (SHPT) we evaluated whether intraoperative parathyroid hormone (iPTH) monitoring is an useful tool as a reference for total parathyroid removal.DesignProspective open single value measurement efficacy study of one intraoperative (i.o.) diagnostic monitoring method (iPTH) on a cohort of surgical patients.PatientsAll patients (n = 35) undergoing TP and SCTx at the Department of Surgery, Donostia Hospital from January 2002 to December 2006.Main outcome measuresSerum levels of iPTH during surgery and prediction time of the of descent of PTH levels (measured in the clinic, at admission day and intra-operatively during induction of anesthesia, and every 5 and 10 minutes after removal of adenoma and 24 hours thereafter) were analyzed.ResultsiPTH levels dropped clearly at ten minutes in all 35 patients and were non-measurable at 24 hours. iPTH decreased from pathological (1302.24 + 424.9 pg/ml) to half (50%) the values at the third intra-operative determination — minute 10 − (614.8 ± 196.62) and was undetectable at 24 hours.ConclusionsIntra-operative measurement of iPTH is useful in the prediction of complete removal of all parathyroid tissue prior to autotransplantation thus avoiding persistence because of incomplete surgery.

Expresión de proteínas relacionadas con resistencia a múltiples fármacos (MDR-proteínas) en tumores sólidos

Resumen Las causas de que una celula tumoral sea resistente a la quimioterapia son muchas y de variada naturaleza. El motivo del presente trabajo es realizar una revision y una puesta al dia de una de estas posibles causas, en concreto, la expresion de proteinas relacionadas con la resistencia a multiples farmacos.