Protocolo para la valoración anatomopatológica de las piezas de cirugía de carcinomatosis peritoneal de origen ovárico, tubárico y peritoneal / Protocol for the examination of surgical specimens from patients with peritoneal carcinomatosis originating in ovary, fallopian tube and peritoneum

La carcinomatosis peritoneal (CP) es una entidad tumoral con una alta tasa de morbimortalidad, considerada la evolución común de varias neoplasias abdominopélvicas, entre ellas, el carcinoma de ovario, trompa uterina y peritoneo. Aunque muchos de estos tumores son sensibles a quimioterapia sistémica, el pronóstico es desfavorable por la elevada tasa de recurrencia. La cirugía de citorreducción (CC) se emplea como tratamiento de primera línea en los estadios avanzados, ya que aumenta la supervivencia de los pacientes cuando la CC es óptima. El procedimiento terapéutico descrito por Sugarbaker para el carcinoma de colon en la década de los 80, que incluye CC y quimioterapia intraperitoneal ha sido adaptado a la CP de origen ginecológico. El estudio anatomopatológico de esta cirugía empieza a ser una práctica habitual en algunos de nuestros servicios. Es un procedimiento complejo, que requiere especialización y sistematización para valorar un gran número de piezas quirúrgicas, cuantificando de forma lo más objetiva posible la carga tumoral. El objetivo de este trabajo es mostrar la experiencia inicial en nuestro servicio de anatomía patológica con pacientes diagnosticadas de CP de origen ovárico, tubárico y peritoneal y sometidas a cirugía citorreductora, destacando el papel del patólogo. Mostramos el esquema de trabajo utilizado en nuestro servicio y resumimos los resultados iniciales de 31 pacientes intervenidas entre enero de 2013 y julio de 2014

Peritoneal carcinomatosis (PC) is a malignant entity with a high rate of morbimortality. It is considered an end-stage common to several abdominal and pelvic malignant tumours, such as epithelial ovarian, fallopian tubal and peritoneal cancer. Although many of these tumors have a good response to chemotherapy, prognosis is poor due to the high rate of recurrence. Surgeons, gynecologists and oncologists are increasingly concerned with improving the survival. The surgical technique described by Sugarbaker in the eighties is a plausible option. It aims for a complete resection of macroscopic carcinomatosis (cytoreductive surgery) followed by intraoperative or perioperative intraperitoneal chemotherapy. This therapeutic option necessarily involves specific multidisciplinary units; histopathology of specimens from this surgical technique is now more frequent in our department. We describe our initial experience with PC originating from epithelial ovarian, tubal and peritoneal cancer treated with the modified Sugarbaker surgery employed in our hospital. We outline our protocol designed to achieve uniformity in procedure, and summarize the initial results

[Protocol for the examination of surgical specimens from patients with peritoneal carcinomatosis originating in ovary, fallopian tube and peritoneum]. / Protocolo para la valoración anatomopatológica de las piezas de cirugía de carcinomatosis peritoneal de origen ovárico, tubárico y peritoneal.

Peritoneal carcinomatosis (PC) is a malignant entity with a high rate of morbimortality. It is considered an end-stage common to several abdominal and pelvic malignant tumours, such as epithelial ovarian, fallopian tubal and peritoneal cancer. Although many of these tumors have a good response to chemotherapy, prognosis is poor due to the high rate of recurrence. Surgeons, gynecologists and oncologists are increasingly concerned with improving the survival. The surgical technique described by Sugarbaker in the eighties is a plausible option. It aims for a complete resection of macroscopic carcinomatosis (cytoreductive surgery) followed by intraoperative or perioperative intraperitoneal chemotherapy. This therapeutic option necessarily involves specific multidisciplinary units; histopathology of specimens from this surgical technique is now more frequent in our department. We describe our initial experience with PC originating from epithelial ovarian, tubal and peritoneal cancer treated with the modified Sugarbaker surgery employed in our hospital. We outline our protocol designed to achieve uniformity in procedure, and summarize the initial results.

Mantle Cell Lymphoma with Central Nervous System Involvement Simulating Bilateral Subdural Hematomas.

We describe a 71-year-old-patient receiving antiplatelet therapy and being attended by emergency medical services for psychomotor retardation and gait disturbance. An emergency computed tomographic scan showed a bilateral subacute hematoma. The patient reported a fall 2 weeks earlier. We performed bilateral drills and saw a solid mass that was biopsied. The patient had a history of mantle cell lymphoma (MCL) in complete remission (results of bone marrow biopsy and whole-body positron emission tomography-computed tomography scans were normal 6 months earlier). We diagnosed an intracranial MCL by immunohistochemistry, flow cytometry, and fluorescence in situ hybridization. We performed magnetic resonance imaging. The results of a new bone marrow biopsy were positive for recurrence of MCL. MCL constitutes approximately 5%-6% of non-Hodgkin lymphoma. The incidence of central nervous system (CNS) involvement between MCLs is 4.1%. After a review of the literatures we found small series comprising 3-5 cases and a multicenter study with 57 cases. Until now, the median survival was 3.7 months. Ibrutinib, an oral Bruton tyrosine kinase inhibitor, has demonstrated efficacy and CNS penetration in relapsed or refractory MCL with rapid and complete response even after 1 year of follow-up. Our patient received ibrutinib and had a complete response at 3 months, which was maintained to the present (6 months). After a review of the literature, we found different pathologies that can mimic subdural hematomas. However, this is the first report of a lymphoma with CNS involvement mimicking bilateral subdural hematomas. This report contributes to the knowledge of lymphomas with CNS involvement. Its strange radiographic appearance and histologic type make it unique.

Primary Malignant Melanoma of the Esophagus: A Case Report and Review of the Literature.

BACKGROUND Primary malignant melanoma of the esophagus (PMME) is a rare cancer with a poor prognosis. It is often difficult to differentiate from non-epithelial malignant tumors, and immunohistochemical staining may be needed to diagnose the condition. The mainstay of treatment is usually surgical with curative or palliative intent, since radio- and chemotherapy do not really improve the outcome. The average survival rate after surgery is 34.5 months. At the time of diagnosis, 40-80% of cases have local regional lymph node metastases. CASE REPORT The case of a 67-year-old male patient with PMME is reported. He presented with progressive dysphagia. A computerized tomography was performed in which a polypoid mass was observed in the distal esophagus. It was originally suspected to be an adenocarcinoma, but was subsequently correctly diagnosed by immunohistochemical staining with HMB-45 antibody and by the presence of S-100 protein. A subtotal esophagectomy was performed. CONCLUSIONS Very few cases of PMME have been reported in the literature and there is only limited clinical experience with this disease. Therefore, it is very difficult to establish clear criteria for clinical recognition of this type of melanoma. Early histopathological confirmation of the character is essential for further treatment in case of confirmation of malignancy.

Multicentric recurrent parotid pleomorphic adenoma in a child

Mixed tumours of the parotid gland are rare in childhood and recurrence of this tumour is infrequent. Some authors report a higher rate of recurrence with some histological subtypes, like hypocellular variant. Female sex and young age at initial treatment are also risk factor for recurrence. Also the first surgical treatment, tumoure nucleation or parotidectomy, has been implicated as a cause for recurrence. We present a case of a multicentric doubly recurrent parotid pleomorphic adenoma, 7 and 14 years after tumour enucleation, in a 9-year-old child. All the nodules resected showed the hypocellular variant of pleomorphic adenoma. We consider the relationships between the choice of treatment, the histologic subtypes of pleomorphic adenomas and multifocal tumours and recurrence. In this case, we believe the recurrence was related to surgical enucleation previously performed and the hypocellular histological subtype. We conclude that tumour enucleation is a risk factor for recurrence and total parotidectomy is the treatment of choice for pleomorphic parotid adenoma also in childhood (AU)

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Multicentric recurrent parotid pleomorphic adenoma in a child.

Mixed tumours of the parotid gland are rare in childhood and recurrence of this tumour is infrequent. Some authors report a higher rate of recurrence with some histological subtypes, like hypocellular variant. Female sex and young age at initial treatment are also risk factor for recurrence. Also the first surgical treatment, tumour enucleation or parotidectomy, has been implicated as a cause for recurrence. We present a case of a multicentric doubly recurrent parotid pleomorphic adenoma, 7 and 14 years after tumour enucleation, in a 9-year-old child. All the nodules resected showed the hypocellular variant of pleomorphic adenoma. We consider the relationships between the choice of treatment, the histologic subtypes of pleomorphic adenomas and multifocal tumours and recurrence. In this case, we believe the recurrence was related to surgical enucleation previously performed and the hypocellular histological subtype. We conclude that tumour enucleation is a risk factor for recurrence and total parotidectomy is the treatment of choice for pleomorphic parotid adenoma also in childhood.

Transformation of vulvar pseudolymphoma (lymphoma-like lesion) into a marginal zone B-cell lymphoma of labium majus.

Lymphomas arising in the female genital tract are extremely uncommon. Diffuse large B-cell lymphoma and follicular lymphoma are the most common types. We describe the case of an 80-year-old woman with a recurrent lesion in the vulva initially diagnosed as a lymphoma-like lesion and evolving 7 years later into a marginal zone B-cell lymphoma (lymphoplasmacytic lymphoma). Diagnosis was based on the monotypic pattern of the plasmacellular component and the clonal rearrangement of immunoglobulin heavy chain genes. No previous cases of vulvar marginal zone B-cell lymphoma arising in the context of a persistent lymphoma-like lesion have been reported. We highlight the importance of differentiating benign from malignant lymphoid infiltrates in the vulva.