[Rectal cancer and Trousseau syndrome. Case report]. / Síndrome de Trousseau y cáncer de recto. Informe de un caso.

BACKGROUND: The Trousseau syndrome, first described in 1865, is the relationship of venous thromboembolisms and cancer. We present a case with rectal cancer and Trousseau syndrome. CLINICAL CASE: Female 40 years old, went to the Coloproctology Service for painless bleeding. A computed tomography report showed a tumor of 5 by 6 cm up 5 cm from the anal margin. Ultra-low anterior resection with colonic reservoir and loop ileostomy surgery was performed. The pathology report showed a semidiferenciate adenocarcinoma of the rectum and we established the stage as T3N0M0. Within 72 hours of her operation, she experienced sudden hypotension and painful abdominal distention. A second surgery was done finding necrosis of the colon from the splenic angle until the colonic reservoir with thrombi in the left colic artery, ischemic signs of bilateral fallopian tubes, ovaries, uterus, pelvic floor and the small intestine, 40 cm before ileostomy and ileon. Left hemicolectomy and colostomy was done. She was taken to intensive care where continuous administration of heparin was given; she died within 5 days because of multiorgan failure. CONCLUSIONS: The mechanism for this syndrome was unknown but there are several hypotheses, suggesting that hematological cancer patients are at an increased risk of deep vein thrombosis. Pancreatic cancer is the most common presentation with this syndrome (in 50% of cases). We suggested continuing with the standards of prevention of thromboembolism.

antecedentes: el síndrome de Trousseau se describió por primera vez en 1865; es la relación entre tromboembolismo venoso y cáncer. Objetivo: informar el caso de una paciente con cáncer de recto y síndrome de Trousseau. Caso clínico: paciente femenina de 40 años de edad que acudió al servicio de Coloproctología por rectorragia indolora. La TAC reportó un tumor de 5 por 6cm y del margen anal a 5cm. Se efectuó resección anterior ultrabaja, con reservorio colónico e ileostomía de protección. El reporte de patología fue de: adenocarcinoma semidiferenciado del recto, con clasificación T3N0M0. A las 72 horas del postoperatorio tuvo hipotensión arterial súbita y distensión abdominal dolorosa. En la reintervención quirúrgica se encontró: necrosis del colon desde el ángulo esplénico hasta el reservorio colónico, con trombos en meso, signos de isquemia en el útero, trompa de Falopio y ovarios, piso pélvico y 40 cm de intestino delgado, antes de la ileostomía e íleon. Se realizó hemicolectomía izquierda y colostomía. Se trasladó a la unidad de terapia intensiva donde continuó con la administración de heparina; falleció a los cinco días por insuficiencia multiorgánica. Conclusiones: el mecanismo de este síndrome se desconoce pero existen varias hipótesis: se ha sugerido que los cánceres hematológicos son los que tienen mayor riesgo de trombosis venosa profunda. El cáncer de páncreas se relaciona con este síndrome en 50% de los casos. Se sugiere continuar con las normas de prevención del tromboembolismo.

Mucinous cystadenoma of the appendix. Case report.

BACKGROUND: Pathology of the appendix represents >50% of surgical activity. It is necessary to consider rare pathologies such as mucoceles. These have a high frequency in females with a F/M ratio of 4:1, as well as in patients >50 years of age. The objective of this study is to report on a case of mucinous cystadenoma of the appendix. CLINICAL CASE: We present the case of a 34-year-old male with a history of juvenile rheumatoid arthritis diagnosed at 9 years of age. At 22 years of age, the patient underwent bilateral hip arthroplasty. Since 1998, the patient has presented with renal amyloidosis and has been under steroid treatment to date. The current problem evolved 1 year ago during control studies for his disease. Computed tomography (CT) of the abdomen was done, demonstrating a tumor in the right iliac fossa. Colonoscopy confirmed a tumor with smooth edges located in the cecum. Surgery was recommended with a diagnosis of lipoma of the cecum. During surgery, a large tumor was found, indicating a poor prognosis. Right hemicolectomy was done. Surgical specimen was sent to pathology with a report of mucinous cystadenoma. CONCLUSIONS: The group of symptoms of cystadenoma is nonspecific. Diagnostic methods include X-rays, ultrasound, CT and colonoscopy. Mucinous cystadenoma is the most common form of mucocele of the appendix. Diagnostic protocols are the same for benign appendix pathology. Treatment is surgical and type of surgery depends on the size of the mucocele. Mucoceles of the appendix are pathologies with a favorable prognosis when appropriate treatment is done.

Cistoadenoma mucinoso de apéndice: informe de un caso / Mucinous cystadenoma of the appendix: case report

Introducción: La patología apendicular representa más de 50% de la actividad quirúrgica general, y dentro de ésta hay que considerar las raras, entre las que se incluyen los mucoceles, cuya incidencia es mayor en mujeres (4/1) y en pacientes con edad superior a 50 años. El objetivo de este trabajo es informar un caso de cistoadenoma mucinoso de apéndice. Caso clínico: Hombre de 34 años de edad con diagnóstico a los nueve años de edad de artritis reumatoide juvenil; con prótesis bilateral de cadera a los 22 años. Presenta amiloidosis renal desde 1998 y se encuentra en tratamiento con esteroides hasta el momento de este informe. El cuadro clínico se inició un año atrás, cuando en una tomografía axial computarizada de abdomen de control se encontró tumoración en la fosa iliaca derecha, confirmada por colonoscopia en ciego. Por diagnóstico presuntivo de lipoma fue programado para cirugía; en el transoperatorio se encontró gran tumoración de pronóstico reservado, por lo que se decidió hemicolectomía derecha. El informe de patología de la pieza quirúrgica fue cistoadenoma mucinoso. Conclusiones: La sintomatología del cistoadenoma es inespecífica. Los métodos de diagnóstico incluyen rayos X, ultrasonido, tomografía axial computarizada y colonoscopia. El cistoadenoma es la forma más común de los mucoceles apendiculares y el protocolo de diagnóstico es igual que para patología de apéndice cecal. El tratamiento de elección es la cirugía y depende del tamaño del mucocele. El pronóstico es bueno con el tratamiento adecuado.

BACKGROUND: Pathology of the appendix represents >50% of surgical activity. It is necessary to consider rare pathologies such as mucoceles. These have a high frequency in females with a F/M ratio of 4:1, as well as in patients >50 years of age. The objective of this study is to report on a case of mucinous cystadenoma of the appendix. CLINICAL CASE: We present the case of a 34-year-old male with a history of juvenile rheumatoid arthritis diagnosed at 9 years of age. At 22 years of age, the patient underwent bilateral hip arthroplasty. Since 1998, the patient has presented with renal amyloidosis and has been under steroid treatment to date. The current problem evolved 1 year ago during control studies for his disease. Computed tomography (CT) of the abdomen was done, demonstrating a tumor in the right iliac fossa. Colonoscopy confirmed a tumor with smooth edges located in the cecum. Surgery was recommended with a diagnosis of lipoma of the cecum. During surgery, a large tumor was found, indicating a poor prognosis. Right hemicolectomy was done. Surgical specimen was sent to pathology with a report of mucinous cystadenoma. CONCLUSIONS: The group of symptoms of cystadenoma is nonspecific. Diagnostic methods include X-rays, ultrasound, CT and colonoscopy. Mucinous cystadenoma is the most common form of mucocele of the appendix. Diagnostic protocols are the same for benign appendix pathology. Treatment is surgical and type of surgery depends on the size of the mucocele. Mucoceles of the appendix are pathologies with a favorable prognosis when appropriate treatment is done.

Perianal nodular hidradenocarcinoma. Case report.

BACKGROUND: Eccrine glands (sweat glands) appear in all sites of the skin and are more abundant in hands and feet. Nodular hidradenocarcinoma (NH) is a rare malignant and aggressive tumor of the eccrine glands. The objective of this study is to report a case of perianal hidradenocarcinoma. CLINICAL CASE: We present the case of a 75-year-old female with diabetes, hypertension, and hypothyroidism. Physical examination revealed a small perianal tumor that was palpated near the anal canal. Biopsy was done. Pathology report revealed perianal hidradenoma. Rectosigmoidoscopy was normal. Computed tomography showed 1-cm adenopathies in inguinal and right iliac regions. Extensive resection of the tumor was done. Definitive pathology report was malignant eccrine acrospiroma. The patient underwent adjuvant chemotherapy in the Oncology Service and died 1 month later. CONCLUSIONS: In patients with a first symptom of metastases in the inguinal region, suspicion must be directed to the anal canal.

Hidradenocarcinoma nodular perianal: informe de un caso / Perianal nodular hidradenocarcinoma: case report

Introducción: Las glándulas ecrinas (sudoríparas) se encuentran en todos los sitios de la piel y son más abundantes en las palmas de las manos y plantas de los pies. El hidradenocarcinoma nodular es un tumor raro de las glándulas ecrinas, maligno y agresivo. El objetivo de este trabajo es informar de un caso de hidradenocarcinoma perianal. Caso clínico: Mujer de 75 años de edad, diabética, hipertensa e hipotiroidea. Al examen físico se observó y palpó tumoración perianal pequeña que llegaba hasta canal anal; se tomó biopsia con resultado de hidradenoma perianal. Se llevó a cabo rectosigmoidoscopia hasta 25 cm, normal. Tomografía computarizada: adenopatías de 1 cm en región inguinal e iliaca derecha. Se realizó resección amplia de la tumoración. El informe de patología fue acrospiroma ecrino maligno. Actualmente la paciente se encuentra en tratamiento complementario con quimioterapia. Conclusiones: En pacientes que debuten con lesiones malignas metastásicas en región inguinal debe pensarse en origen del conducto anal.

BACKGROUND: Eccrine glands (sweat glands) appear in all sites of the skin and are more abundant in hands and feet. Nodular hidradenocarcinoma (NH) is a rare malignant and aggressive tumor of the eccrine glands. The objective of this study is to report a case of perianal hidradenocarcinoma. CLINICAL CASE: We present the case of a 75-year-old female with diabetes, hypertension, and hypothyroidism. Physical examination revealed a small perianal tumor that was palpated near the anal canal. Biopsy was done. Pathology report revealed perianal hidradenoma. Rectosigmoidoscopy was normal. Computed tomography showed 1-cm adenopathies in inguinal and right iliac regions. Extensive resection of the tumor was done. Definitive pathology report was malignant eccrine acrospiroma. The patient underwent adjuvant chemotherapy in the Oncology Service and died 1 month later. CONCLUSIONS: In patients with a first symptom of metastases in the inguinal region, suspicion must be directed to the anal canal.

Externalization of a soft tissue chordoma. Case report.

BACKGROUND: Chordomas are rare, slow-growing tumors that originate in the primitive notochord. The most frequent location is the sacrococcygeal region. It is more frequent in males. Average age is 61 years old. The aim of this study is to report a case of externalization of a chordoma in the sacral region. CLINICAL CASE: We present the case of a 62-year-old female whose disease began 4 years before admission, reporting discomfort in the sacral region. In the intergluteal area, the presence of a mass that increased in size during the previous year was noticed and palpated. On proctological examination, a tumor of 6 cm in diameter is found located in the midsacral region 3 cm from the anal canal. On palpation, the tumor is hard and painless with a regular and mobile surface. X-ray of the coccyx was normal. Surgery was performed with total excision and primary closure. Histopathological report was chordoma. DISCUSSION: Theoretically, notochord in the vertebral bodies is not completely degenerative and the remaining tissue persists, producing chordomas. The most common symptom is local pain. Diagnosis is made by imaging: x-ray of lumbosacral spine, computed tomography and magnetic resonance imaging. The treatment of choice is wide surgical resection. CONCLUSIONS: Diagnosis of chordoma is rare but should be kept in mind in patients with pain in lower back or coccyx and, even more so, if accompanied by a mass with years of evolution.

[Giant epidermoid presacral and retrorectal cyst: case report]. / Quiste epidermoide gigante del espacio presacro y posanal.

BACKGROUND: The presacral space, which contains different types of embryonic tissue, is a potential site for several tumors including epidermoid cyst. Presacral cysts are divided into two major groups: teratomas and developmental cysts. Presacral developmental cysts are rare congenital injuries with significant manifestations in the adult. Our objective was to report a case of a giant epidermoid presacral and retrorectal cyst. CLINICAL CASE: We present the case of a 28-year-old female whose clinical feature was the presence of a perianal mass and difficult micturition of 8 years. Rectosigmoidoscopy and videocolonoscopy were normal. Simple and contrast abdominal and pelvic tomography (CT) were ordered as well as nuclear magnetic resonance (NMR) imaging to determine extension and location of the tumor. These studies demonstrated three tumors in the presacral space. Surgery using a combined abdominal and perianal approach was done. Pathological report was epidermoid cysts. The patient had a favorable evolution with no reports of fecal incontinence. CONCLUSIONS: The accepted definition describing epidermoid cyst is squamous stratified epithelium with keratohyaline grains, but with no other skin structures. Developmental cysts are slow growing due to their unique location, despite being congenital. Diagnosis is confirmed by ultrasound, CT, and NMR. Biopsy is contraindicated. Three described approaches are abdominal, combined or abdominoposterior, and transsacral, each with its specific indications.

Quiste epidermoide gigante del espacio presacro y posanal / Giant epidermoid presacral and retrorectal cyst: case report

Introducción: El espacio presacro, el cual contiene algunos tipos de tejidos embrionarios, es un sitio potencial para varios tumores, siendo el quiste epidermoide uno de ellos. Las lesiones quísticas presacras se dividen en dos grupos: teratomas y quistes del desarrollo, estos últimos son lesiones congénitas raras y sus manifestaciones en el adulto son excepcionales. El objetivo de la presente investigación es informar el caso de un quiste epidermoide gigante del espacio presacro y posanal. Caso clínico: Mujer de 28 años con cuadro clínico que se inició ocho años atrás con masa tumoral perianal y dificultad para la micción. La rectosigmoidoscopia y videocolonoscopia fueron normales. Se solicitó tomografía abdominopélvica simple y contrastada, así como resonancia magnética nuclear, para determinar extensión y localización de la tumoración, con las cuales fue posible apreciar tres tumoraciones en el espacio presacro. Se realizó cirugía por abordaje combinado: abdominal y perianal. El examen patológico indicó quistes epidermoides. La paciente evolucionó favorablemente sin datos de incontinencia fecal. Discusión: La descripción aceptada de quiste epidermoide es que posee epitelio escamoso estratificado con gránulos queratohialinos, pero no con otras estructuras de piel. Los quistes del desarrollo son formaciones de crecimiento lento que por su especial localización se manifiestan de forma tardía a pesar de ser congénitos. El diagnóstico se realiza por ultrasonido, tomografía axial computarizada y resonancia magnética nuclear. La biopsia preoperatoria está contraindicada. Se describen tres vías de abordaje: abdominal, combinada o abdominoposterior y transacra, cada una con sus indicaciones.

BACKGROUND: The presacral space, which contains different types of embryonic tissue, is a potential site for several tumors including epidermoid cyst. Presacral cysts are divided into two major groups: teratomas and developmental cysts. Presacral developmental cysts are rare congenital injuries with significant manifestations in the adult. Our objective was to report a case of a giant epidermoid presacral and retrorectal cyst. CLINICAL CASE: We present the case of a 28-year-old female whose clinical feature was the presence of a perianal mass and difficult micturition of 8 years. Rectosigmoidoscopy and videocolonoscopy were normal. Simple and contrast abdominal and pelvic tomography (CT) were ordered as well as nuclear magnetic resonance (NMR) imaging to determine extension and location of the tumor. These studies demonstrated three tumors in the presacral space. Surgery using a combined abdominal and perianal approach was done. Pathological report was epidermoid cysts. The patient had a favorable evolution with no reports of fecal incontinence. CONCLUSIONS: The accepted definition describing epidermoid cyst is squamous stratified epithelium with keratohyaline grains, but with no other skin structures. Developmental cysts are slow growing due to their unique location, despite being congenital. Diagnosis is confirmed by ultrasound, CT, and NMR. Biopsy is contraindicated. Three described approaches are abdominal, combined or abdominoposterior, and transsacral, each with its specific indications.

[Complementary thermo-obliteration in double anal fistula]. / Termoobliteración complementaria en fístulas anales dobles.

BACKGROUND: Anal fistula is an abnormal tract or cavity originating in a cryoglandular infection, whose primary internal opening is in the anorectal junction. Secondary orifice is located in the perianal skin, perineum or vagina. We undertook this study to use an electrosurgery complementary procedure that treats multiple tracts without sectioning the anal sphincter. METHODS: We reviewed the files of 1354 patients who were operated on for anal fistula in the Regional Hospital of the Ecuadorian Institute of Social Security, Guayaquil, Ecuador, from January 1978 to December 2002. We selected only 79 patients with diagnoses of double anal fistula. Study design was descriptive, retrospective, and longitudinal. RESULTS: Seventy one patients were male (89.8%). The age average was 42.5 years. The symptomatic period had an average of 14 months. The period of wound healing and incapacity from work was 3.3 weeks. The procedure failed in seven patients (8.8%) and eight patients presented partial incontinence to gases (10.12%). DISCUSSION: Fulguration is a coagulation method in which the active electrode is maintained near the tissue (1-10 mm distance) and the energy dissipated in the area by means of sparks. Fulguration has greater penetration capability and reaches a greater degree of dehydration of the tissue than electrocoagulation. Factors that influence recurrence and anal incontinence are, for recurrence: type of fistula, horseshoe extension, identification of the primary orifice, previous anal surgery and surgeon's experience. Incontinence has been related to female gender, high anal fistulas, and type of previous surgery (fistulectomy/fistulotomy). CONCLUSIONS: In complex double anal fistulas, it is preferable to resect the tract and use complementary thermo-obliteration. In this way, anal continence is not altered substantially.

Angioqueratoma circunscrito interglúteo: reporte de un caso / Intergluteal angiokeratoma circumscriptum: report of one case

Introducción: el término angioqueratoma se aplica a un conjunto de enfermedades con una base clínica de lesiones vasculares comunes. El objetivo de este reporte es comunicar el hallazgo de un angioqueratoma interglúteo. Caso clínico: hombre de 26 años quien desde la infancia presentó tumoración interglútea, manejada en varias ocasiones sin resultados. Cuando acudió a la consulta que motivó este reporte, presentaba tumoración verrucosa interglútea dolorosa y sangrante de 7 x 4 cm, de contornos irregulares. Se efectuó resección quirúrgica amplia. Los resultados histopatológicos indicaron angioqueratoma circunscrito con bordes libres de tumor. Discusión: de los cinco tipos de angioqueratomas descritos en la literatura (corporal difuso, de Mibelli, de Fordyce, solitario y corporal circunscrito), en nuestro caso se identificó angioqueratoma circunscrito por las características físicas y anatomopatológicas de la tumoración. El diagnóstico diferencial debe establecerse con el hemangioma verrucoso y la enfermedad de Fabry tipo II (angioqueratoma corporal difuso). Conclusiones: la patogénesis del angioqueratoma es desconocida y el tratamiento recomendado es el quirúrgico.

BACKGROUND: The term angiokeratoma is applied to several diseases with cutaneous vascular lesions. CLINICAL CASE: A 26-year-old male presented with an intergluteal tumor from childhood until the present time, which was treated on several occasions without results. The patient presented to the hospital complaining of a painful and bleeding intergluteal tumor. DISCUSSION: Five types of angiokeratomas have been described (angiokeratoma corporis diffusum, angiokeratoma of Mibelli, angiokeratoma of Fordyce, solitary angiokeratoma and angiokeratoma corporal circumscriptum). This patient was identified with an angiokeratoma circumscriptum by pathological and physical characteristics of the tumor. Differential diagnosis is with verrucous hemangioma and the Fabry disease type II (angiokeratoma corporis diffusum). CONCLUSION: Pathogenesis is unknown and surgery is the recommended treatment.

[Intergluteal angiokeratoma circumscriptum. Report of one case]. / Angioqueratoma circunscrito interglúteo. Reporte de un caso.

BACKGROUND: The term angiokeratoma is applied to several diseases with cutaneous vascular lesions. CLINICAL CASE: A 26-year-old male presented with an intergluteal tumor from childhood until the present time, which was treated on several occasions without results. The patient presented to the hospital complaining of a painful and bleeding intergluteal tumor. DISCUSSION: Five types of angiokeratomas have been described (angiokeratoma corporis diffusum, angiokeratoma of Mibelli, angiokeratoma of Fordyce, solitary angiokeratoma and angiokeratoma corporal circumscriptum). This patient was identified with an angiokeratoma circumscriptum by pathological and physical characteristics of the tumor. Differential diagnosis is with verrucous hemangioma and the Fabry disease type II (angiokeratoma corporis diffusum). CONCLUSION: Pathogenesis is unknown and surgery is the recommended treatment.

[Synergistic necrotizing fasciitis of the anoperineal and external genitalia: progression to Fournier's gangrene?]. / Fascitis sinérgica necrotizante de ano perineal y genitales externos. Es adecuada esta denominación o se debe continuar con la de gangrena de Fournier?

INTRODUCTION: In 1883, Fournier, a French venereal disease physician, described five patients with fulminant surgical infection of the male genitalia. He pointed to three main features: 1) sudden onset, 2) rapid gangrene progression, and 3) no visible cause. Since then, multiple primary causes have been identified such as genitourinary infections, local trauma, different surgical procedures or instruments, as well as acute anorectal disease (ischiorectal abscesses). We describe the experience with patients diagnosed with Fournier's Syndrome or necrotizing anoperineal and genital infection in our hospital service. MATERIAL AND METHODS: Medical files were reviewed from the Proctology Department of the IESS Regional Hospital from January 1, 1980, to May 15, 2004. The method was descriptive, retrospective, and longitudinal. RESULTS: Fifty-one predominantly male patients (48 males and 3 females) with a mean age of 49 years were studied. Symptoms were present for 8.7 days. The most common associated disease was diabetes mellitus (24 patients). Cultures were only used for anaerobes and mainly E. coli was identified. Extensive incisions, continuous debridement and necrotic tissue were included in the surgical procedure as many times as necessary. There was progression in the abdominal wall and the inguinal region. Orchidectomy and diversion colostomy were performed in 10 patients. Average hospital stay was 32 days. Antibiotic treatment was used against aerobes and anaerobes. Eight patients died (15.5%). CONCLUSIONS: In these patients, local resistance is disturbed and cell immunity is impaired. Also, the nutritional and vascular condition of patients is affected because of aerobe/anaerobe bacterial synergy. It is recommended to immediately start pus drainage, incise even hyperemic tissue, and debride and resect necrotized tissue several times to avoid residual infection and sepsis.