Impact of the type of anthracycline and of stem cell transplantation in younger patients with acute myeloid leukaemia: Long-term follow up of a phase III study

We provide a long-term evaluation of patients enrolled in the EORTC/GIMEMA AML-10 trial which included a total of 2157 patients, 15-60 years old, randomized to receive either daunorubicin (DNR, 50 mg/m²), mitoxantrone (MXR, 12 mg/m²), or idarubicin (IDA, 10 mg/m²) in addition to standard-dose cytarabine and etoposide for induction chemotherapy and intermediate dose cytarabine for consolidation. Younger patients who reached complete remission with complete (CR) or incomplete (CRi) recovery were then scheduled to receive an allogeneic hematopoietic stem cell transplantation (HSCT). That was if they had a HLA-identical sibling donor; in all other cases, an autologous HSCT had to be administered. At an 11-year median follow-up, the 5-year, 10-year and 15-year overall survival (OS) rates were 33.2%, 30.1% and 28.0%, respectively. No significant difference between the three randomized groups regarding OS was observed (P = .38). In young patients, 15-45 years old, no treatment difference (P = .89) regarding OS was observed, while in patients 46-60 years old, MXR and IDA groups had a trend for a longer OS as compared to the DNR group (P = .029). Among younger patients without a favorable MRC cytogenetic risk subgroup who achieved a CR/CRi after induction chemotherapy, those with a HLA-identical sibling donor had higher 10-year and 15-year OS rates than those without. In older patients who reached CR/CRi, the long-term outcomes of those with or without a donor was similar. In conclusion, long-term outcomes of the study confirmed similar OS in the three randomized groups in the whole cohort of patients.     

Myeloid sarcoma with megakaryoblastic differentiation mimicking a sellar tumor

Myeloid sarcoma (MS) is a localized extra‐medullary tumor mass of immature myeloid cells, arising de novo or related to acute myeloid leukemia, of which it can be a forerunner, a coinciding or late event. Less commonly, MS represents an acute blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. This rare condition commonly consists of a proliferation of more or less immature cells with a myeloid immunophenotype, very exceptional cases showing a megakaryoblastic or erythroid differentiation. The most common localization of MS is the skin, lymph node, soft tissues and bones, but CNS involvement is exceedingly rare, with no cases reported in the sellar region. We report a 54‐year‐old man, affected by myeloproliferative neoplasm, JAK2 V617F‐positive of 13 years duration, who acutely presented with a third cranial nerve palsy; neuroradiology documented a space‐occupying lesion at the level of the sellar, upper clival and right parasellar regions, that was sub‐totally removed with a trans‐sphenoidal approach. The histological examination documented a proliferation of large, blastic cells, frequently multinucleated; a diagnosis of MS with megakaryoblastic differentiation, arising in a background of chronic idiopathic myelofibrosis, was suggested by immunohistochemistry, owing to CD42b, CD45, CD61 and LAT (linker for activation of T cells) positivity. In addition, homozygous JAK2 V617F mutation was detected from the myeloid sarcoma specimen. A few weeks after surgery, an acute blastic leukemic transformation occurred and, despite chemotherapy, the patient died 2 months after surgery. To the best of our knowledge, this is the first MS case with megakaryoblastic differentiation arising within the CNS.     

Ackerman's tumor (verrucous carcinoma) of the larynx. A clinicopathologic study of 77 cases

A series of 77 cases of verrucous carcinoma of the larynx is presented. The tumor is relatively rare and the present series has been selected from 2,398 primary and recurrent malignant neoplasms of the larynx and hypopharynx evaluated at the Department of Otolaryngology of Padua University during the period from January 1966 to December 1978. The median age of patients was 58 years. The tumor appears to be exophytic, broadly implanted and fungating in aspect, with papillary fronds and a locally invasive character. It is composed of highly differentiated epithelial squamous cells covered by a thick keratinized layer arranged in deeply invaginated folds. Cellular response in tumor stroma is marked, and the neoplasm has a low-grade malignancy. Close cooperation between the pathologist and the clinician is needed in order to establish the nature of the lesion, which looks clinically malignant but may appear histologically benign. Benign responses such as hyperkeratosis, acanthosis, or the presence of a benign papillomatous area necessitate further investigation with another biopsy examination from deeper layers. The elective treatment is surgery, as irradiation may cause anaplastic transformation with metastatic spread. Neck dissection is not indicated as this laryngeal tumor has so far never metastasized to cervical lymph nodes or to other organs.     

Pattern of Gastric Emptying in Functional Dyspepsia. An Ultrasonographic Study

We assessed the ultrasonographic pattern of gastric emptying in patients with functional dyspepsia, evaluating its relationship with symptoms. Twenty dyspeptic patients, with slight (group A) and severe (group B) symptoms, and 10 controls (group C) underwent ultrasonographic study of gastric emptying by measuring postprandial variations of the antral area at regular intervals. The time at which the antral area returned to the basal value was assumed to be the final emptying time. The final emptying time was significantly longer in both group A (294 +/- 42 min) and group B (340 +/- 36 min) compared to controls (244 +/- 21 min), but no significant difference was observed between them. A significantly (P < 0.05) greater dilation of the antral area was found at 50 min in group B compared to group A patients. A different pattern of antral motor function rather than a delay of gastric emptying correlates with the severity of symtoms in dyspeptic patients.