Forty patients with high-risk hematologic malignancies, median age 9 years, underwent haploidentical-HSCT from April 2005 to April 2015. Seventeen patients were transplanted with CD3-depleted PBSCs by negative selection (TCD group) following a reduced-intensity conditioning regimen (RIC), and 23 patients received T-cell-replete PBSCs followed by post-transplantation cyclophosphamide (PT-Cy group) after myeloablative conditioning (n=16) or RIC (n=7). Outcomes are reported for the TCD and PT-Cy recipients, respectively. Engraftment was achieved in 88% versus 100%. Median time to neutrophils>500/μL was 10 days versus 15 days. Platelets>20 000/μL occurred at a median of 16 days versus 20 days, respectively. Transplant-related mortality (TRM) was 24% versus 26% at 1 year. The cumulative incidence (CI) of grade III-IV acute GvHD was 7% versus 5%, and chronic GvHD 9% versus 53% (P=0.029). Relapse at 2 years was 31% versus 24%. Actuarial overall survival rates at 2 years were 47% versus 48%. Causes of death were infections (n=3), sinusoidal obstructive syndrome (n=4), acute GvHD (n=2) and relapse (n=9). These results indicate that haploidentical-HSCT is feasible in Uruguay. The TRM rate is of concern and should be the focus of continuing attention. Chronic GvHD risk was higher in the PT-Cy approach, so modifications are justified.

A high incidence of nasal complications after conventional transsphenoidal surgery observed through examination and not reported spontaneously point to the need of otorhinolaryngological investigation complemented by nasal endoscopy in patients submitted to procedures through this route.

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There is a lack of clinical studies and reviews specifically evaluating the role of surgical hypopituitarism and radiotherapy in the HQOL, mood, and cognitive function of patients.

Introduction: Acne fulminans is a rare disease, which most often affects teenagers and young adults, being described associated with exogenous testosterone use and with elevated adrenal androgens in congenital adrenal hyperplasia. We describe a case of acne fulminans in a toddler with adrenocortical carcinoma. Clinical Case: A 2.3 years-old male diagnosed with adrenocortical tumor presenting pubarche, increased penis and body hair, severe acne lesions in face, chest and back and also arterial hypertension. He was previously healthy, with normal development at his first year. The investigation showed adrenal hyperandrogenism and hypercortisolism. Serum values of Total Testosterone >1500 ng/dL (<7,00 - 25,91 ng/dL), ACTH: 7,2 pg/mL (<46 pg/mL), DHEAS: 682 µg/dL (<15 µg/dL), Aldosterone: 11 ng / dL (2.5-39.2 ng /dL), Serum Cortisol 8AM: 30.65µg/dL (5.27 to 22.45 µg/dL), Salivary midnight cortisol: 29.5nmol/L (11:30PM-00:30AM < 7.6 nmol/L). The abdominal computed tomography (CT) showed the left suprarenal gland with a 5,1 x 3,9 cm lesion. Left adrenalectomy was performed, whose histology confirmed adrenocortical carcinoma, without exceeding the capsule, but with a vascular invasion focus and Ki-67 rate of 20%. Hormonal levels in the early follow up were normal and prophylactic corticosteroid therapy was progressively removed. At the three months after surgery outpatient follow-up, worsening of his skin acneiform lesions was observed. He presented painful papules, pustules and crusts at face, chest and back, with purulent exudation and bleeding lesions, associated with axillary and inguinal adenomegaly and acne fulminans was diagnosed. Clinical and ultrasonographic examination also showed the presence of hepatosplenomegaly,. The treatment was initially made with oral corticosteroids and antibiotics. At this time, laboratory tests of androgens, DHEAS and salivary cortisol were normal, but inflammatory markers were high. A new postoperative abdominal CT showed, at the left adrenal topography, two lesions, 47HU attenuation, 1.5 x 1.2 x 1.1cm and 2.3 x 1.7 x 2.0cm, and also confirmed mild hepatosplenomegaly. Biopsy of inguinal lymph nodes was performed, and histology showed lymphoid follicles hyperplasia. Conclusions: This case report showing the association between acne fulminans and adrenocortical carcinoma, at postoperative time, when the child already had normal serum androgens and after hypercortisolism resolution, without oral corticosteroid therapy. Disordered immune response and/or hypersensitivity reaction to Propionibacterium acnes antigens, are also considered as possible triggering factors, although the etiology for this cases is not well established. The description of an acne fulminans case in such a young patient with adrenocortical carcinoma seems unusual and such association deserves further elucidation.