[Galli-Galli disease presenting as lichenoid papules in the flexures]. / Presentación de enfermedad de Galli-Galli como pápulas liquenoides flexurales.

Galli-Galli disease is a rare genodermatosis currently regarded as an acantholytic variant of Dowling-Degos disease. The 2 diseases have the same clinical features: reticular hyperpigmented macules in the great skin folds, erythematous scaly papules and plaques, comedo-like lesions, and pitted perioral scars, and the only differentiating characteristic is the histological finding of acantholysis, usually without dyskeratosis. We describe the case of a patient with hyperpigmented papules in the skin folds as the only sign of Galli-Galli disease, and we present a review of the literature.

Impact of novel histopathological factors on the outcomes of liver surgery for colorectal cancer metastases.

INTRODUCTION: We evaluated the impacts of a series of novel histopathological factors on clinical-surgical outcomes and survival of patients who underwent surgery for colorectal cancer liver metastasis, with and without neoadjuvant chemotherapy. MATERIALS AND METHODS: A prospective database including 150 consecutive patients who underwent 183 hepatic resections for metastatic colorectal cancer was evaluated. Among them, 74 (49.3%) received neoadjuvant chemotherapy before surgery. The histopathological factors studied were: a) microsatellitosis, b) type and pattern of tumour growth, c) nuclear grade and the number of mitoses/mm(2), d) perilesional pseudocapsule, e) intratumoural fibrosis, f) lesion cellularity, g) hypoxic-angiogenic perilesional growth pattern, and h) the tumour normal interface. RESULTS: Three or more metastatic lesions, R1 resection margins, and <50% tumour necrosis were prognostic factors for a worse OS, but only the former was confirmed to be an independent prognostic factor in the multivariate analysis. Furthermore, tumour fibrosis <40% and cellularity >10% were predictive of a worse neoadjuvant therapy response, but these findings were not confirmed in the multivariate analysis. Finally, tumour necrosis <50%, cellularity >10%, and TNI >0.5 mm were prognostic factors for a worse DFS and AS in the univariate but not in the multivariate analysis. CONCLUSIONS: Several factors seem to influence the outcomes of surgery for colorectal cancer liver metastasis, especially the number of the lesions, the margins of resection, the percentage of necrosis and fibrosis, as well as the cellularity and the TNI.

[Malignant fibrous histiocytoma of soft tissue: description of 10 cases]. / Histiocitoma fibroso maligno de partes blandas: descripción de 10 casos.

We analyze the clinical and histological features of 10 cases of malignant fibrous histiocytoma of soft tissue. Nine belonged to the pleomorphic-verticillate variety and one was myxoid. The initial clinical feature was a palpable mass in all cases except three with retroperitoneal localization, where constitutional symptoms predominated. After therapy (surgery in all, associated with radiotherapy in four), seven patients had local relapse and two had distant metastases. 50% died, with a mean survival of 13 months. We discuss the prognostic factors and the therapeutic approach, with emphasis on aggressive therapy and the need for radical surgery and postoperative adjuvant therapy.

[Incidence and mortality of laryngeal neoplasms. Soria, 1950-1989]. / Incidencia y mortalidad neoplásica laríngea. Soria, 1950-1989.

Age-adjusted mortality and incidence rates for larynx cancer were calculated in Soria (urban and rural areas), for the 1950-89 and 1981-89 periods respectively. Both mortality and incidence rates were higher in urban area (p less than 0.01), and in males (p less than 0.01). There has been no increase in the mortality trend for the 1950-89 period. Age-adjusted incidence rates for men in Soria are higher respect to those reported in other countries. Soria and Murcia rates are the lowest in Spain larynx cancer records, being Asturias rates the highest in the world (20.6 per 100,000 population).

Recommendations of a group of experts for the pathological assessment of tumour regression of liver metastases of colorectal cancer and damage of non-tumour liver tissue after neoadjuvant therapy.

Colorectal cancer (CRC) incidence has increased during the past decades in Spain, being the first malignant tumour in incidence. Observed mortality for CRC is mainly due to liver and lung metastases. The only curative treatment is surgery; new surgical techniques and neoadjuvant treatments have increased the number of surgery candidate patients. Patients should be managed with a multidisciplinary approach that includes imaging techniques, chemotherapy, surgery and pathological assessment. As an answer to this approach, a group of pathology experts interested on CRC liver metastases aimed to review the diagnosis and prognosis of liver mestastases and developed practical recommendations for its assessment. The expert group revised the current literature and prepared questions to be discussed based on available evidence and on their clinical practise. As a result, recommendations for the assessment of tumour regression of liver metastases are proposed, which could be implemented in oncology centres allowing assessment standardisation for these patients. Prospective multi-center studies to evaluate these recommendations validity will further contribute to improve the standard care of CRC liver metastases patients.

[Bilateral angiomyolipomas of the kidney in Bourneville's tuberous sclerosis]. / Angiomiolipomas renales bilaterales en la esclerosis tuberosa de Bourneville.

We report on a 25-year-old patient diagnosed as having Bourneville tuberous sclerosis with a giant angiomyolipoma 16 X 12 cm. in diameter, and two small angiomyolipomas in the left kidney, multiple asymptomatic angiomas in the right kidney and two 1 cm. diameter angiomas in the liver. The presenting features were intense left-sided abdominal pain of sudden onset, frank hematuria, acute anemic syndrome, nausea and vomiting. Patient work up included x-ray of chest, abdomen, and skull, bilateral renal arteriography and ultrasound were diagnostic of Bourneville tuberous sclerosis with multiple bilateral renal angiomyolipomas. The hematologic and hemodynamic compromise and the almost completely absent intact parenchyma in her left kidney did not permit a conservative surgical procedure and the patient was submitted to a left nephrectomy. The patient had the following characteristic features of Bourneville's disease: epilepsy, intracranial calcifications on CT, sebaceous adenomas on face, fibromas under nails of left hand and foot, bony lesions in the form osteosclerosis and chylous pleural effusion. Her intelligence level was normal and no optic nerve phakomas were observed. The literature on angiomyolipoma and Bourneville's tuberous sclerosis is reviewed. The importance of using ultrasound and CT in combination to diagnose and follow up renal angiomyolipomas is highlighted. Treatment of angiomyolipomas must be based on two parameters: symptoms and size. Symptomatic angiomyolipomas warrant angiographic work up and selective arterial embolization or the most conservative surgical procedure possible (enucleation, partial nephrectomy). Asymptomatic angiomyolipomas warrant CT and/or ultrasound examination every 6 or 12 months depending on size.

[Malignant fibrous histiocytoma of the mediastinum]. / Histiocitoma fibroso maligno mediastínico.

Soft tissue sarcomas are neoplasms which envolve from mesodermic tissue, being characterized for their low incidence (1% of all malignant tumors), histologic heterogeneity and diverseness regarding their localization. We present a new case of this infrequent tumor: malignant fibrous histiocytoma of exceptional localization: mediastinum.