[Primary non-Hodgkin's lymphoma of the breast. A case report]. / Linfoma no Hodgkin primario de la glándula mamaria. Reporte de un caso.

BACKGROUND: Primary breast lymphomas, a rare subtype of non-Hodgkin's lymphoma, represent 0.04 to 0.5% of all breast cancers, 0.38 to 0.7% of all lymphomas, and 1.7 to 2.2% of extranodal lymphomas. The treatment choice is based on chemotherapy containing anthracycline and rituximab. Surgery is limited to being less invasive and only for diagnostic purposes. Radiotherapy has an important role as consolidation therapy, particularly in patients with negative nodes. CLINICAL CASE: A 70 year old woman with a breast nodule in the left upper outer quadrant, with slow growth, expansive, painless, and accompanied by skin changes, malaise, weight loss, fatigue, chill, and sweating. There was tissue replacement by the mammary gland tumour, skin changes due to invasion, and a 5cm axillary lymphadenopathy. The mammography showed skin thickening and a dense pattern of 80% of breast tissue replacement, and the lymphadenopathy with loss of radiolucent centre and soft tissue invasion. The biopsy confirmed a diffuse high grade large cell lymphoma. She received an Rituximab (R-CHOP) chemotherapy scheme and radiotherapy with tangential and supraclavicular and axillary fields. After completing the chemotherapy, the patient is on follow-up, and at 15 months she is alive without disease activity. CONCLUSIONS: Primary lymphoma of the breast is a rare entity. Multimodal treatment with combined chemo-radiotherapy is the cornerstone. Surgery is reserved only for diagnostic purposes.

[Diagnostic of uterine sarcoma, review of 11 cases]. / Diagnóstico de sarcoma uterino, revisión de 11 casos.

BACKGROUND: Uterine sarcomas are rare but extremely aggressive malignant uterine pathology; mostly incidentally diagnosed at an advanced stage, with a higher aggressiveness and poor prognosis. OBJECTIVE: To determine the epidemiological profile of women with this diagnosis in Ginecopediatria Hospital, UMAE 48 of IMSS and its correlation with the literature. MATERIAL AND METHODS: A retrospective and observational study was done from 2007 to 2014 in UMAE 48, IMSS León, Guanajuato, in which the clinical records of patients with such diagnoses were reviewed. RESULTS: A total of 11 cases with age range 21-66 years had abnormal bleeding (82%), abdominal pain (64%), enlarged uterus (64%) and isolated elevation of lactic dehydrogenase (55%). The leiomyosarcoma accounted for 55%, sarcoma mixed Mullerian (SMM) 27% and endometrial stromal sarcoma (SEE) in 22% of cases. The stage I was the most frequent (55%) and histological type, which presented a more advanced stage was the SMM (stage IIIC). The hysterectomy with pelvic and paraaortic lymphadenectomy was the most common treatment (67%). The 45% of patients had tumor activity subsequent to treatment and the use of radiotherapy and chemotherapy was not associated with activity. CONCLUSION: Clinical features, risk factors and distribution of the presentation of the various sarcomas were according with previous studies, requiring further research to improve prognosis and survival in this type of tumor.

[Proctocolectomy with ileoanal anastomoses and desmoid tumor treated with resection. One case of familial adenomatous polyposis]. / Proctocolectomía con anastomosis íleo-anal y resección de tumor desmoide. Un caso de poliposis adenomatosa familiar.

BACKGROUND: Familial adenomatous polyposis (FAP) is a rare disease caused by a mutation in the adenomatous polyposis coli gene (APC). CASE REPORT: We report the case of a 32-year-old woman, with abdominal pain and increased abdominal perimeter, as well as melena and weight loss. She had a tumor of 12 cm in diameter in the right iliac fossa. After the administration of contrast media we found the abdominal tumor compatible with sarcoma versus desmoid tumor. We performed a colonoscopy and we found colorectal polyps. The biopsy reported tubulovillous adenomas. A panendoscopy showed polyps in fundus and body of stomach; the state of the duodenum was normal. Tumor resection was performed with abdominal wall reconstruction with mesh and restorative proctocolectomy with ileoanal reservoir and a temporary ileostomy. The histopathology report demonstrated an abdominal wall desmoid tumor and identified 152 tubulovillous polyps which affected all the portions of colon and rectum. CONCLUSIONS: FAP is an autosomal dominant disease caused by a mutation in the APC gene which results in the development of multiple colorectal polyps. Described in 1991 the APC gene is located at chromosome region 5q21. Without prophylactic surgery, virtually all patients develop colorectal cancer in the third decade of life. Desmoid tumors and duodenal polyps are now the leading cause of death in patients with FAP.

Introducción: la poliposis adenomatosa familiar (PAF) es una rara enfermedad causada por una mutación en el gen de la poliposis adenomatosa coli (APC). Caso clínico: mujer de 32 años, con dolor y aumento del perímetro abdominal además de evacuaciones melénicas y pérdida de peso. La paciente presentó un tumor de 12 cm de diámetro en la fosa iliaca derecha. Tras la administración de medio de contraste, en una tomografía se apreció el tumor abdominal con reforzamiento compatible con sarcoma frente a tumor desmoide. Se realizó colonoscopia, por medio de la que se encontraron pólipos en el recto y el colon. La biopsia reportó adenomas túbulo-vellosos. Una panendoscopía demostró pólipos en fondo y cuerpo gástrico; el duodeno se encontraba en estado normal. Se realizó resección del tumor en pared abdominal y reconstrucción con malla además de proctocolectomía restaurativa con un reservorio íleo-anal con una ileostomía temporal. Se reportó tumor desmoide en la pared abdominal y se identificaron 152 pólipos túbulo-vellosos que afectaban todas las porciones del colon y el recto. Conclusiones: la PAF es una enfermedad autosómica dominante causada por una mutación en el gen APC que da como resultado el desarrollo de múltiples pólipos tanto en el colon como en el recto. Descrito en 1991, el gen APC se localiza en el cromosoma 5q21. Sin cirugía profiláctica, todos los pacientes desarrollarán cáncer colorrectal en la tercera década de la vida. Los tumores desmoides y los pólipos duodenales son ahora la causa de muerte en los pacientes con PAF.

[Isolated splenic metastases from cervical cancer: a rare entity]. / Metástasis esplénicas aisladas por cáncer cervicouterino: una rara entidad.

BACKGROUND: Splenic metastases from solid tumors are a rare event with an incidence of only 2.9% to 9%. Splenic metastases from cervical cancer are a rare entity. Only a few cases have been reported of isolated spleen metastases from cervical cancer. CLINICAL CASE: We present the case of a 76-year-old woman with moderately differentiated endocervical adenocarcinoma stromal and endocervical invasion. Clinical stage was Ib1 and Ca-125 values of 150 U. She was managed with hysterectomy and pelvic lymphadenectomy. She received pelvic radiotherapy (45 Gy) followed 24 Gy of brachytherapy. Two years later she presented with abdominal pain. Abdominal computed tomography showed two splenic parenchymal lesions without disease in the remainder of the abdominal cavity and chest with a Ca-125 of 2,733 U. The patient is submitted to splenectomy. Histopathology demonstrates splenic metastases of well-differentiated adenocarcinoma from the endocervix. Immunohistochemical stain showed positivity from carcinoembryonic antigen; estrogen and progesterone receptors are negative. Ca-125 level 8 weeks after surgery was 16 U/ml. The patient received six cycles of adjuvant chemotherapy with paclitaxel and cisplatin. At 12 months follow-up the patient is alive and without evidence of tumor activity. CONCLUSIONS: The spleen is an uncommon site of metastasis. Splenectomy is considered the appropriate treatment in order to avoid complications such as splenic rupture and splenic vein thrombosis as well as to improve pain control from splenomegaly. Twelve months after surgery our patient is alive and without evidence of tumor activity.

Antecedentes: las metástasis al bazo por tumores sólidos son excepcionales, su incidencia es apenas de 2.9 a 9%. Las metástasis esplénicas por cáncer cervicouterino son infrecuentes, solo se han reportado algunos casos. Caso clínico: paciente femenina de 76 años de edad, con adenocarcinoma de endocervix moderadamente diferenciado; con invasión al estroma cervical y al canal endocervical, etapa clínica Ib1; el Ca-125 de 150 U. Se trató con histerectomía y linfadenectomía pélvica. Recibió radioterapia pélvica 45 Gy, seguida de braquiterapia 24 Gy. Dos años después tuvo dolor abdominal y, en una tomografía, se encontraron dos lesiones parenquimatosas esplénicas sin existir enfermedad en el resto de la cavidad abdominal y el tórax, tiene elevación del Ca-125 de 2,733 U. Se le realizó esplenectomía; el reporte histopatológico demostró metástasis esplénicas de adenocarcinoma bien diferenciado del endocervix. El estudio de inmunohistoquímica mostró positividad al antígeno carcinoembrionario, los receptores de estrógeno y progesterona resultaron negativos. El Ca-125 de 16 U/mL a ocho semanas de la cirugía. Recibió tratamiento adyuvante, seis ciclos con paclitaxel y cisplatino. A 12 meses de seguimiento la paciente está viva y sin actividad tumoral. Conclusiones: el bazo es un sitio poco común de metástasis. La esplenectomía se ha considerado el tratamiento apropiado para evitar complicaciones como: ruptura esplénica, trombosis de la vena esplénica y disminución del dolor originado por la esplenomegalia. Doce meses después de la cirugía la paciente está viva y sin evidencia de actividad tumoral.

[Carcinosarcoma of the breast a rare entity with fatal prognosis. One case report]. / Carcinosarcoma mamario una rara entidad con pronóstico fatal. A propósito de un caso.

BACKGROUND: breast metaplastic carcinomas are a heterogeneous group of neoplasms that exhibit a poor prognosis compared with invasive ductal carcinoma. Correspond less than 1% of all malignant neoplasms of the mammary gland. They usually present as high-grade tumors with a lower rate of lymph node metastases and decreased expression of estrogen and progesterone receptors and Her2 and increased expression of Her1 and Ki-67. CLINICAL CASE: we report a 52 year old woman with a breast carcinosarcoma presented with a left breast tumor fungated, ulcerated, polypoid and 18 cm in major diameter with lymph node metastases at diagnosis. She received multimodal management with neoadjuvant chemotherapy, followed by mastectomy and adjuvant chemotherapy; she presented progression of the disease with lung metastases and local massive recurrence, eventually died from complications associated to the disease. CONCLUSIONS: metaplastic carcinomas of the breast are extremely rare entities. Due the nature of disease and presentation, the prognosis is poor in these patients. There are several histologic subtypes based on studies of hematoxylin and eosin and immunohistochemical stains. It requires multimodal therapy (surgery, radiotherapy and chemotherapy) for best results.

Antecedentes: los carcinomas metaplásticos de la glándula mamaria son un grupo heterogéneo de neoplasias con pobre pronóstico en comparación con el carcinoma ductal invasor. Corresponden a menos de 1% del total de las neoplasias malignas de la glándula mamaria. Casi siempre se manifiestan como tumores de alto grado con menor índice de metástasis ganglionares y menor expresión de receptores de estrógeno y progesterona, Her2 y mayor expresión de Her1 y Ki-67. Caso clínico: se reporta el caso de una mujer de 52 años de edad con carcinosarcoma mamario que se manifiesta con un tumor mamario izquierdo, fungado, ulcerado, polipoide, de 18 cm de diámetro, con metástasis ganglionares al momento del diagnóstico. Recibió tratamiento multimodal con quimioterapia neoadyuvante seguida de mastectomía y quimioterapia complementaria; evolucionó con metástasis pulmonares y recurrencia local masiva. Falleció por complicaciones derivadas de la enfermedad.Conclusiones: los carcinomas metaplásticos de la glándula mamaria son padecimientos sumamente raros. Debido a la naturaleza de la enfermedad y su forma de presentación el pronóstico es pobre. Existen varios subtipos histológicos basados en estudios de hematoxilina y eosina o tinciones de inmunohistoquímica. El tratamiento debe ser multimodal (cirugía, radioterapia y quimioterapia) para obtener mejores resultados.

[Cardiac tamponade due to pericardial metastases of an unknown primary tumor]. / Taponamiento cardiaco por metástasis pericárdicas originadas de tumor primario desconocido.

BACKGROUND: the pericardiocenthesis is indicated as treatment of cardiac tamponade. The prevention of recurrences can be achieve by pericardial instillation of sclerosing agents, radionuclide agents as well as external beam radiotherapy that had given good results in radiosensitivity tumors. Pericardial window, pleuropericardiotomy, pericardiectomie and percutaneous pericardiotomie, are used when the pericardiocenthesis cannot be carried out. CLINICAL CASE: a female patient, 34 years with cardiac tamponade was attended in the emergency room due to hemopericardium and pericardial metastases of unknown primary tumor. She was managed with pericardial window and systemic chemotherapy. After two year, she is alive without evidence of pericardial effusion recurrence and without evidence of tumor activity. CONCLUSIONS: pericardiocentesis is an indication in cardiac tamponade. The multimodal treatment provides better results in patients with metastases to the pericardium and cardiac tamponade. It includes local and systemic chemotherapy and radiation therapy.