Carcinoma epidermoide metastásico asociado a epidermólisis ampollosa distrófica / Metastasic squamous cell carcinoma in dystrophic epidermolysis bullosa

Caso clínico: femenino de 18 años con diagnóstico de epidermolisis ampollosa distrófica (EAD) quién desarrolló una neoformación nodular sobre una úlcera crónica. Se diagnosticó carcinoma epidermoide (CE) invasor al que se realizó resección. Sin embargo, 5 meses después del tratamiento quirúrgico presentó metástasis a ganglios, pulmón e hígado con desenlace fatal. Comentarios: el CE es la causa más importante de muerte en pacientes con EAD. Suele ser agresivo y metastásico. Se recomienda una vigilancia cada 3 a 6 meses para realizar diagnóstico y tratamiento oportunos (AU)

Case report: 18-year-old female patient with dystrophic epidermolysis bullosa (DEB) who developed a tumor over a chronic ulcer. She was diagnosed with invasive squamous cell carcinoma (SCC) and underwent surgical resection. However, 5 months later she presented metastases to the lymph nodes, lung and liver with a fatal outcome. Comments: SCC is the most important cause of death in patients with DEB. It is usually aggressive and metastatic. Surveillance every 3 to 6 months is recommended for prompt diagnosis and treatment (AU)

[Intra-abdominal desmoplastic small round cell tumour]. / Tumor intraabdominal desmoplásico de células pequeñas y redondas.

BACKGROUND: The desmoplastic small round cell tumour is a rare and aggressive intra-abdominal neoplasia, with only 200 cases reported, and a higher incidence in men and predilection for the second decade of life. Histologically characterized by the presence of small nests of undifferentiated tumour cells, wrapped in fibrous desmoplastic stroma. CLINICAL CASE: A 24 year old male started with abdominal pain of 4 weeks onset in the right upper quadrant, colic type, sporadic, self-limiting and accompanied by early satiety, decreased appetite, and involuntary weight loss of 10 kg in 3 months. At the time of admission the abdomen was globular, with decreased peristalsis, soft, depressible. Computed tomography of the abdomen showed multiple enlarged lymph nodes in the abdominal-pelvic cavity. A laparotomy was performed, with a subsequent omentum resection due to the presence of multiple tumours, which microscopically were characterised by groups of small, round, blue cells, separated by a desmoplastic stroma. The immunohistochemistry was positive for desmin (> 75%), epithelial membrane antigen (> 75%), CD99 (> 50%), and S100 (25%), concluding with an abdominal tumour of small, round, blue cells as a diagnosis. Chemotherapy treatment was initiated based on IMAP plus GM-CSF. CONCLUSIONS: The desmoplastic small round cell tumour is a rare neoplasia, with diagnostic complexity and a lethal course. Its clinical presentation is unspecific. Histologically, it is classified as an aggressive soft tissue sarcoma that shares similar characteristics with the family of the small and blue cells tumours.

[Bilateral ovarian Burkitt's lymphoma. A case presentation]. / Linfoma tipo Burkitt bilateral de ovario. Presentación de un caso.

BACKGROUND: Burkitt lymphoma, is described as an aggressive form of non-Hodgkin lymphoma of B cells which occurs most often in children and young adults, ovarian lymphoma can appear as a primary lesion or more commonly referred to as a metastasis. Primary ovarian lesions are rare manifestations corresponding to 0.5% of non-Hodgkin lymphoma and 1.5% of ovarian tumors. Clinic case: 31 years old female with general weakness, march incapacity, dyspnea, hyporexia, fever, diaphoresis, weight loss of 20 kg, flat abs with abdominal pain; Ca125 610 U/ml. Abdominal computed tomography shows a solid aspect tumor which affects the right pelvic cavity. Bilateral ovarian tumors were removed. Microscopically, both lesions show a "starry sky" pattern composed by a monotonous infiltration of lymphocytes mixed with large and clear macrophages, several atypical mitoses, and necrosis and hemorrhage areas. Immunohistochemistry was positive for CD10, CD20, and negative for CD3 and high Ki67 proliferation index. Bilateral ovarian Burkitt's lymphoma was diagnosed. CONCLUSION: Bilateral ovarian Burkitt's lymphoma is a rare entity, with a variability of presentations, the abdominal pain and abdominal tumors are the most frequent. The patient's prognosis at short term is poor, therefore it's necessary to know this entity and make an early diagnosis.

Antecedentes: el linfoma de Burkitt es una forma agresiva de los linfomas no Hodgkin de células B que ocurre con mayor frecuencia en niños y adultos jóvenes; el linfoma de ovario puede aparecer como lesión primaria o, más comúnmente, como una metástasis. Las lesiones primarias de ovario son manifestaciones raras que corresponden a 0.5% de los linfomas no Hodgkin y 1.5% de los tumores de ovario. Caso clínico: paciente femenina de 31 años de edad, con debilidad generalizada, incapacidad para la marcha, disnea, hiporexia, fiebre, diaforesis, pérdida de 20 kg de peso, abdomen plano, con dolor abdominal; Ca125 610 U/mL. La tomografía computada abdominal mostró un gran tumor de aspecto sólido que afectaba a la cavidad pélvica derecha. Se extirparon los tumores ováricos bilaterales. Desde el punto de vista microscópico ambas lesiones muestran una imagen en "cielo estrellado" compuesta por un patrón de infiltrado monótono de linfocitos mezclada con macrófagos de citoplasma amplio y claro, abundantes mitosis atípicas, zonas de necrosis y hemorragia. La inmunohistoquímica reveló positividad para CDI0 y CD20, negativo con CD3, índice de proliferación Ki67 alto. Se diagnosticó linfoma tipo Burkitt bilateral de ovario. Conclusiones: el linfoma de Burkitt de ovario bilateral es poco frecuente, con variabilidad de presentación; el dolor abdominal y los tumores abdominales son lo más frecuente. El pronóstico a corto plazo de las pacientes es malo, por lo que es necesario conocer esta afección para poder establecer el diagnóstico temprano.