Prenatal diagnosis of truncus arteriosus with interrupted aortic arch and abnormal limbs due to an umbilical cord amniotic band: rare entities with an unusual association.

Truncus arteriosus, interrupted aortic arch and amniotic band syndrome are rare conditions. We report a case of a 38-year-old pregnant woman who was diagnosed on a routine morphological ultrasound scan with a Van Praagh type A4 persistent truncus arteriosus with an aortic arch interruption type B and abnormal limbs (oedematous left hand, hypoplastic fingers on the right hand and right big toe amputation). Elective termination of the pregnancy was carried out and the pathological examination confirmed all the sonographic findings. Furthermore, an amniotic band parallel to the umbilical cord undetected during the ultrasound scans was revealed, and was entangled around the right hand, left wrist and the umbilical cord, causing strangulation. We present the unusual association of these independent pathologies and emphasise the usefulness of fetal autopsy in all cases of pregnancy termination and abnormal ultrasound findings to make the complete diagnosis.

Posttransplant Lymphoproliferative Disease and Inhibitors of Mammalian Target of Rapamycin: When a Quick Look Back Can Change the Perspective.

Posttransplant lymphoproliferative disease represents a heterogeneous group of diseases characterized by uncontrolled proliferation of lymphocytes, favored by immunosuppression. Several risk factors for its development have been described, with Epstein-Barr virus infection being a main cause of early-onset forms and chronic antigen stimulation of donors and/or accumulated immunosuppression as key factors of later forms of lymphocyte transformation. The present clinical case presents a patient diagnosed with posttransplant lymphoproliferative disease 3 years after renal transplant who had a potentially lethal complication related to conversion to inhibitors of mammalian target of rapamycin. Because clinical studies that establish the most suitable treatment are lacking, it is recommended to identify the strategy, defining possible risks versus benefits of conversion to inhibitors of mammalian target of rapamycin in cases of posttransplant lymphoproliferative disease, and to maintain a high level of surveillance in case of possible secondary effects that can be verified after their introduction.

Hemólisis masiva en un caso de sepsis por Clostridium perfringens / Fatal hemolysis due to Clostridium perfringens sepsis

Presentamos un caso de hemólisis masiva fulminante a causa de una sepsis por Clostridium perfringens en un paciente diabético que acude a un hospital comarcal manifestando dolor abdominal. Mostramos las imágenes de la sangre periférica obtenida antes de la muerte del paciente, que tuvo lugar 22 h después de su ingreso hospitalario (AU)

We report a case of fulminant massive hemolysis due to Clostridium perfringens sepsis in a diabetic patient who was seen in a local hospital complaining of abdominal pain. The patient died 22 h after hospital admission. We present images of peripheral blood taken before death (AU)

Hemorragia alveolar como causa de muerte en paciente con crioglobulinemia mixta asociada a VHC / Alveolar hemorrhage as a cause of death in a patient with mixed cryoglobulinemia associated with HCV

Presentamos el caso de una mujer de 80 años con hepatopatía crónica secundaria a infección por virus de la hepatitis C (VHC) y linfoma primario marginal esplénico. Ingresó por episodios de descompensación cardiaca siendo éxitus por insuficiencia respiratoria. Se le diagnosticó crioglobulinemia mixta tipo II asociada a infección por VHC debido a la presencia de crioglobulinas en sangre. El estudio necrópsico reveló hallazgos cutáneos y renales compatibles con esta entidad y determinó la presencia de infiltración de diferentes órganos por linfoma. La causa de muerte fue una hemorragia alveolar pulmonar bilateral masiva. La crioglobulinemia mixta asociada a virus de la hepatitis C (CMVHC) es una entidad reconocida que puede presentar complicaciones, entre las que destacan la afectación renal y la pulmonar. La hemorragia pulmonar es una de las complicaciones más graves, con un alto índice de mortalidad(AU)

An 80 year old woman, who had cronic liver disease secondary to a hepatitis C virus (HCV) infection and primary splenic marginal zone lymphoma, was admitted to hospital with heart failure. The presence of cryoblobulins in the blood led us to diagnose type II mixed cryoglobulinemia associated with HCV (HVCMC). The autopsy revealed cutaneous and renal findings related to HVCMC and lymphoma infiltration was observed in different organs. The cause of death was massive bilateral alveolar haemorrhage. HVCMC is a recognized entity which may have different complications, especially in the lung and kidney; pulmonary hemorrhage is one of the most serious, with a high mortality rate(AU)

Cytological diagnostic clues in fine needle aspiration of breast myofibroblastoma: a case report.

Myofibroblastoma of the breast is a rare, benign mesenchymal tumor derived from the stroma and tends to occur mainly in middle-aged to elderly men. There are a few cases reported in the literature describing its cytological features. We report the cytological and histological findings of a breast myofibroblastoma in a 68-year-old man. He presented with a palpable nodule of 2-months duration in his left breast. The cytological smears obtained by fine needle aspiration showed less cohesive cellularity in a fascicular arrangement consisting of spindle cells with elongated cytoplasm, occasional nuclear grooves, and intranuclear cytoplasmic inclusions associated to fragments of stromal collagen. The cytological diagnosis was a mesenchymal proliferation suggesting the possibility of myofibroblastoma which was confirmed on histopathological examination. The breast myofibroblastoma has distinctive cytological features that in conjunction with the clinical and radiological data allow a specific diagnosis.

Tumor de Brenner benigno bilateral asociado a cistadenoma mucinoso. Una aproximación a los tumores de células transicionales del ovario / Bilateral benign Brenner tumour associated with a mucinous cystadenoma. A case report and a reflection on transitional cell tumours of the ovary

Los tumores de células transicionales del ovario constituyen un raro grupo de tumores epiteliales ováricos que, desde su primera descripción en 1932, han sido sujeto de numerosos trabajos para establecer su histogénesis y comprender su actividad endocrina. Comprenden dos categorías clínico-patológicas: los tumores de Brenner (benigno, borderline o proliferante y maligno) y el carcinoma de células transicionales. Presentamos un caso de tumor de Brenner benigno bilateral y de grandes dimensiones asociado a cistadenoma mucinoso en una mujer de 54 años con antecedentes de carcinoma de mama. Discutimos las principales características clínico-patológicas de este grupo de tumores epiteliales ováricos y su asociación con los tumores mucinosos(AU)

Transitional cell tumours constitute a rare group of ovarian epithelial neoplasms, first described in 1932 and still the subject of many studies to determine their histogenesis and endocrine activity. They are classified into two distinct clinicopathological categories: Brenner tumours (benign, borderline or proliferative and malignant) and transitional cell carcinoma. We report a case of a large, bilateral benign Brenner tumour associated with mucinous cystadenoma in a 54 year old woman with a history of breast carcinoma. The main clinical and pathological features of transitional cell ovarian tumours are described and their association with mucinous tumours is discussed(AU)

Infiltración perineural por células fúngicas. Presentación de un caso y revisión de la literatura / Perineural spread by fungal cells. Case report and literature review

La importancia de la infiltración perineural por células fúngicas se ha infravalorado como factor pronóstico, sobre todo en micosis que se asientan en el área rino-órbito-cerebral y pulmonar. Presentamos el caso de un varón de 73 años diabético y con leucemia mielomonocítica crónica agudizada, que debutó con tumefacción de hemicara izquierda, ocupación de los senos paranasales, extensión de la afección a las partes blandas y fistulización a cavidad bucal. La clínica también denotó afectación cerebral. En las secciones histopatológicas y en las técnicas de histoquímica realizadas, se observó una importante infiltración perineural por células fúngicas. A pesar de un diagnóstico rápido y tratamiento de choque, el paciente falleció. Alertamos que la infiltración perineural puede ser un mecanismo de propagación retrógrado de las micosis, sobre todo las rino-órbito-cerebrales, con extensión al sistema nervioso central(AU)

The perineural spread by fungal cells during the progression of the infection could be an important prognostic factor, especially in mycoses localized in the rhino-orbito-cerebral and pulmonary areas. We present a clinical case of a 73-year-old male, with diabetes and acute myelomonocytic leukaemia that began with tumefaction on the left side of his face, spreading to the sinus with invasion of the soft tissues and fistulae in the oral cavity. Clinical examination showed cerebral involvement. The histopathological sections and the histochemical techniques showed perineural involvement by fungal cells. Although the patient was diagnosed and treated in a short period of time, he died due to the infection. We would like to alert that perineural spread could be a retrograde way of mycoses dissemination, particularly in infections located in areas rich in neural cells(AU)

[Perineural spread by fungal cells. Case report and literature review]. / Infiltración perineural por células fúngicas. Presentación de un caso y revisión de la literatura.

The perineural spread by fungal cells during the progression of the infection could be an important prognostic factor, especially in mycoses localized in the rhino-orbito-cerebral and pulmonary areas. We present a clinical case of a 73-year-old male, with diabetes and acute myelomonocytic leukaemia that began with tumefaction on the left side of his face, spreading to the sinus with invasion of the soft tissues and fistulae in the oral cavity. Clinical examination showed cerebral involvement. The histopathological sections and the histochemical techniques showed perineural involvement by fungal cells. Although the patient was diagnosed and treated in a short period of time, he died due to the infection. We would like to alert that perineural spread could be a retrograde way of mycoses dissemination, particularly in infections located in areas rich in neural cells.

Cardiomegalia masiva debida a carcinoma de células escamosas de la mucosa oral: descripción de un caso de autopsia / Massive cardiomegaly due to squamous cell carcinoma of oral mucosa: report of an autopsy case

IntroducciónLos tumores de cabeza y cuello producen metástasis a distancia en estadios avanzados. La afectación cardiaca por estos tumores es poco habitual, siendo el origen más frecuente en estos casos los tumores localizados en la lengua.Caso clínicoMujer de 58 años con gran cardiomegalia debida a metástasis de un carcinoma de células escamosas originado en la mucosa yugulogingival, que debutó con clínica inespecífica de un mes de evolución y falleció a los 4 días del ingreso. El estudio necrópsico demostró la afectación cardiaca masiva por carcinoma de células escamosas asociada a metástasis en ambos pulmones, hígado y páncreas, acompañado de tromboembolismo pulmonar, sin recidiva de enfermedad local.DiscusiónPlanteamos la dificultad diagnóstica de las metástasis cardiacas debido a su baja frecuencia y clínica escasa, inespecífica y de presentación tardía.ConclusionesActualmente, las técnicas de imagen y la posibilidad de realizar biopsias cardiacas transvenosas aumentan el número de casos diagnosticados en vida. Sin embargo, aún hoy día la mayoría de estas metástasis continúan diagnosticándose en el estudio necrópsico(AU)

IntroductionTumours of the head and neck tend to metastasize in the late stages of the disease; cardiac metastases are unusual, and when they occur the primary tumour is usually found in the tongue.Case reportA 58 year old woman with cardiac metastasis from a squamous cell carcinoma of the jugular-gingival mucosa was admitted to hospital with non-specific symptoms and died 4 days after admission. The necropsy showed massive cardiac squamous cell carcinoma associated to metastases in lungs, liver, pancreas and pulmonary embolism but no local recurrence of disease.DiscussionCardiac metastases are rare and have non-specific symptoms and are therefore difficult to diagnose.ConclusionsImaging techniques and cardiac biopsies allow more cases to be diagnosed in a living patient, although the majority of cardiac metastases are still diagnosed on necropsy(AU)

Rectal inflammatory polyp in a child: an unusual case of polyparasitism.

In recent years, parasitic diseases have increased especially in non-tropical or subtropical countries. We present a case of an 11-year-old boy from Guinea with a rectal polyp parasitized by Schistosoma haematobium and Trichuris trichiura. This parasitic association had never been reported in our country or in Europe.

Double cervical cyst derived from a single thyroglossal duct tract.

Thyroglossal duct cysts are one of the most common congenital cervical pathologic findings in children. This type of cyst can be located anywhere between the base of the tongue and the sternal manubrium. We report the case of a patient with a double thyroglossal cyst, one located in the hyoid region and the other in the thyroid gland. The 2 cysts were connected by a permeable tract, which confirms that an involution failure of the embryonic remains of the thyroglossal duct was responsible for the development of the cyst. Intrathyroid cysts of the thyroglossal duct, though uncommon, should be included in the differential diagnosis of thyroid cysts in children.