Bilobed perforator free flaps for combined hemitongue and floor-of-the-mouth defects.

INTRODUCTION: Combined hemiglossectomy and floor-of-the-mouth defects need accurate reconstructive planning to restore swallowing and speech function. The aim of this prospective study was to evaluate outcomes of the bilobed design applied to perforator free flaps for combined hemitongue and floor-of-the-mouth defects. PATIENTS AND METHODS: Twelve patients with a mean age of 71 years (range, 60-84) addressed to combined hemiglossectomy and floor-of-the-mouth resection and bilobed-shaped perforator free-flap reconstruction were prospectively enrolled. Defects were classified as follows: type 1, including only the anterior mobile portion of the tongue (n = 3); type 2, involving both mobile tongue and tongue base (n = 6); and type 3, including segmental mandibulectomy combined with a type 1 or type 2 defect (n = 3). The Kruskal-Wallis and Bonferroni post hoc tests were used to compare outcomes. RESULTS: Type 1 defects were reconstructed by three anterolateral thigh (ALT) perforator flaps; type 2 defects were reconstructed by four ALT flaps and two vertical deep inferior epigastric perforator flaps; and type 3 defects were restored by three osteocutaneous fibula flaps. Eleven flaps (91.6%) healed uneventfully, while one (8.4%) suffered a small area of skin necrosis whose revision did not compromise functional results. Six patients achieved normal intelligible speech, five had acceptable intelligible speech and one had unintelligible speech (p = 0.356). Swallowing function was considered normal in eight patients and with mild impairment in four (p = 0.178). Cosmesis resulted excellent in seven patients and good in five (p = 0.855). CONCLUSION: The bilobed-shaped perforator free flaps were shown to be a safe and predictable solution for combined hemitongue and floor-of-the-mouth defects providing optimal aesthetic and functional outcomes.

Mortality from second tumour among long-term survivors of retinoblastoma: a retrospective analysis of the Italian retinoblastoma registry.

Survivors of retinoblastoma (Rb) are at high risk of dying from second malignant tumour. The occurrence of second malignant neoplasm (SMN) and related mortality in a cohort of 1111 cases from the Italian Retinoblastoma Registry was analysed, considering the possible role of both genetic and iatrogenic causes. Rb patients had a greater than 10-fold excess in overall mortality compared with the general population (standardized mortality ratio (SMR) 10.73, 95% CI 9.00-12.80). Their excess risk attributable to cancers other than Rb was 14.93 95% CI 10.38-21.49). Survivors of hereditary Rb had an SMR for all causes of 16.25 (95% CI 13.20-20.00), whereas their SMR for all cancers was 25.72 (95% CI 17.38-38.07). Survivors of unilateral sporadic Rb had an SMR of 4.12 from all cancers (95% CI 1.55-10.98) and a much higher excess for overall mortality (SMR 13.34, 95% CI 10.74-16.56). As expected, survivors of hereditary Rb had higher mortality from cancers of the bone (SMR 391.90, 95% CI 203.90-753.20) and soft tissue (SMR 453.00, 95% CI 203.50-1008.40), small intestine (SMR 1375.50, 95% CI 344.00-5499.70), nasal cavity (SMR 13.71, 95% CI 1.93-97.35) and cancers of the brain and central nervous system (SMR 41.14, 95% CI 13.2-127.55).

Shunt-related abdominal metastases in an infant with medulloblastoma: long-term remission by systemic chemotherapy and surgery.

This is the first reported case of long remission of abdominal metastases spread through a ventriculo-peritoneal shunt in an infant diagnosed, four years ago, at age 1 year and 10 months, to have cerebral medulloblastoma. Two years later, while in second complete remission of his cerebral tumor, he showed abdominal metastases, successfully treated by platinum based chemotherapy and surgery. One year later, a second abdominal relapse and hepatic metastases were treated by doxorubicin administration and surgery. Since then the child remained in continuous complete remission. This unusual favorable outcome can be explained by an extreme responsiveness of the tumor, unprotected by the blood brain barrier, to systemic chemotherapy, particularly to doxorubicin administration. The need for careful surveillance of patients with ventriculo-peritoneal shunts is emphasized. Searching for new tools, such as entrapment of doxorubicin in liposomes, able to overcome the blood-brain barrier and to expose brain tumors to effective drugs, probably represents the best choice for future treatment strategies of CNS tumors.

Cardiac betaARK1 upregulation induced by chronic salt deprivation in rats.

The beta-adrenergic receptor (betaAR) kinase (betaARK1) is a G protein-coupled receptor kinase (GRK) that controls cardiac betaAR signaling via receptor phosphorylation, leading to desensitization. We have observed in mice that chronic isoproterenol administration results in increased myocardial levels of betaARK1 activity, suggesting that adrenergic activation can regulate cardiac betaARK1 expression. Thus, we evaluated left ventricular (LV) betaARK1 levels and activity in response to 3 weeks of a low-sodium (0.05%) diet, which is known to chronically activate the sympathetic nervous system. Wistar-Kyoto rats were subjected to either low or regular sodium (2%) intake. To prove the association of betaARK1 expression and low sodium-induced adrenergic activation, a group of rats was subjected to atenolol treatment (1 mg/kg per day) during the low-sodium diet. LV betaARK1 expression was assessed by protein immunoblotting and betaARK1 activity by in vitro GRK phosphorylation assays. We verified the LV protein levels of GRK5, which is abundantly expressed in the heart. A low-sodium diet reduced body weight and cardiac size so that the heart-to-body weight ratio did not change. On the contrary, low-sodium diet increased by 50% both LV betaARK1 protein (densitometry units: normal sodium, 26.5+/-0.9; low sodium, 35.7+/-1.6; P<0.05) and activity (fmol/mg per minute: normal sodium, 6.49+/-1.17; low sodium, 9.15+/-0.93; P<0.05). Atenolol treatment prevented the increase in both protein expression (low sodium plus atenolol, 27.6+/-5.33, P=NS versus normal sodium) and activity (6.54+/-1.19, P=NS versus normal sodium). GRK5 expression was not affected by a low-sodium diet (17.2+/-0.2 versus 18.4+/-0.4, P=NS). Our data indicate that cardiac betaARK1 is regulated by sympathetic action on betaARs as tested by reducing dietary salt and betaAR blockade.

Radiation late effects in children treated for orbital rhabdomyosarcoma.

BACKGROUND AND PURPOSE: The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects. PATIENTS AND METHODS: Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples. All but three patients, who received 45, 54 and 55 Gy respectively, have been treated by immediate radiation at the dose of 60 Gy, delivered in 2 Gy fractions, five times per week, by cobalt 60 megavoltage equipment. Combined chemotherapy using vincristine and vincristine plus dactinomycin on alternate weeks was also administered as part of induction therapy. RESULTS: An overall survival rate of 94.7% was registered. In our patients the majority of radiation late effects were paid by orbit and ocular adnexa. Side effects to lens and ocular structures were fewer and of low grade. CONCLUSIONS: Radiation therapy is still essential for local control of orbital rhabdomyosarcoma, however radiation side effects have to be carefully considered together with the therapeutic goal to be obtained.

Interstitial 9q deletion is associated with CD7+ acute leukemia of myeloid and T lymphoid lineage.

We report the clinical, hematological and immunophenotypic characteristics from four cases of acute leukemia with interstitial deletion of chromosome 9, ie del(9)(q12-q22), as a single chromosomal abnormality. Three patients had acute myeloblastic leukemia (AML) and one T origin acute lymphoblastic leukemia (ALL). According to FAB classification, blasts were classified as M1 (two patients), M2 (one patient), and L2 (one patient). In two out of three AML cases a myelodysplastic syndrome, one AREB-t and one AREB diagnosed 6 and 11 months before respectively, preceded the onset of AML. Morphological examination showed dysgranulopoiesis, dyserythropoiesis and cytoplasmic vacuoles in two AML patients, while a strong positivity to myeloperoxidases was observed in all AML cases. As concerns immunophenotypic findings, blast cells from two of three AML patients expressed CD7 and CD34, while those from the T-ALL case displayed CD33 and CD34 along with CD7. These observations suggest that del (9q) is associated with CD7+ acute leukemia of myeloid or lymphoid lineage.

Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy.

Primary maxillary localization of Ewing's sarcoma is unusual. Involvement of facial bones is characterized by clinical and radiological features distinct from those commonly observed in other sites. Because of the above peculiarities a delay in diagnosis and thus in starting treatment is very probable in such cases. We report here two new cases of Ewing's sarcoma localized to facial bones, successfully treated by local high dosage radiotherapy and systemic chemotherapy. Our experience suggests that, especially for particular sites not suitable to radical surgery, radiation therapy can represent an effective tool to achieve local control of the tumor.

Granulocytic sarcoma in nonleukemic children: report of two new cases successfully treated by local radiation therapy and systemic chemotherapy.

Granulocytic sarcoma (GS) is a rare tumor composed of immature myeloid cells. Exceedingly rare in childhood, it has more commonly been described in association with acute myeloid leukemia. Occasional nonleukemic patients generally go on to develop overt leukemia in a mean period of 10.5 months from diagnosis of GS. We report here two new cases of GS diagnosed in nonleukemic children. They were successfully treated with local radiation therapy and conventional systemic chemotherapy. The need to suspect more often this all too frequently misdiagnosed disease is emphasized. The role of optimally delivered radiation therapy in achieving and maintaining local control of the tumor is discussed.

Treatment of EBV-induced lymphoproliferative disorder with epipodophyllotoxin VP16-213.

A case of haemophagocytic lymphohistiocytosis consistent with X-linked lymphoproliferative disorder is described. Remission was observed after administration of VP-213, a cytotoxic drug generally used to treat histiocytosis. The child is currently in good clinical health.

A novel chromosomal abnormality detected in a case of acute myelomonocytic leukemia.

A not yet recorded translocation, t(3:17)(q21;q23), detected in a case of acute myelomonocytic leukemia, is reported. In spite of an aggressive cytotoxic chemotherapy, the disease showed a rapid fatal course, confirming the dismal prognostic significance of structural, 'primary' chromosomal abnormalities in acute leukemias.

Familial erythrophagocytic lymphohistiocytosis: adverse prognostic significance of delayed diagnosis.

Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disorder of the monocyte-macrophage system, for which an autosomal recessive mode of inheritance has been postulated. It is characterized by a dismal prognosis and is peculiar of early infancy. Three new cases of infants affected by FEL are reported. All three patients were diagnosed about three months after the onset of symptoms, and all three died shortly after diagnosis. The need for early diagnosis and prompt, intensive cytotoxic chemotherapy is emphasized.

Unusual seizures with a benign course in a case of acute lymphoblastic leukemia of childhood.

We report the unusual seizures in a patient with acute lymphoblastic leukemia. The convulsive disorder began acutely as partial somatomotor status epilepticus and with diffuse EEG slowing. The seizures then became myoclonic-atonic (drop attacks) and an EEG focus appeared on the left paramedian centro-parietal areas, activated by proprioceptive stimuli. Despite the severity of the clinical picture at onset, the seizures showed a benign course, and disappeared on carbamazepine therapy. We think that this epileptic syndrome may have been caused by diffuse iatrogenic encephalopathy, probably related to intrathecal methotrexate therapy.

Iron absorption and iron deficiency in infants and children with gastrointestinal diseases.

Iron status, iron absorption, and intestinal blood loss were studied in 199 children undergoing diagnostic evaluation for suspected malabsorption. Evaluation of iron status included hematological indices, serum ferritin, and transferrin saturation. Iron absorption was assessed by the increment of serum iron after an oral iron load. Iron deficiency was common among patients affected by malabsorptive states, such as celiac disease (84%), cow's milk intolerance (76%), Crohn's disease (72%), and giardiasis (64%), whereas it was less common among patients with postinfectious enteritis (41%) and chronic nonspecific diarrhea (11%). Intestinal blood loss was seen only in patients with Crohn's disease and cow's milk intolerance, irrespective of iron nutritional status. On the other hand, iron malabsorption was very common, affecting 85-95% of the iron-deficient patients in all diagnostic groups, except in chronic nonspecific diarrhea. Iron malabsorption was less common among patients with adequate iron nutritional status than in those with iron deficiency. Iron malabsorption appears to play a major role in the pathogenesis of iron deficiency in patients with malabsorption. The iron absorption test shows greater sensitivity as a screening test for upper intestinal malabsorption than the D-xylose absorption test.

Multidisciplinary treatment of primary orbital rhabdomyosarcoma. A single-institution experience.

Orbital rhabdomyosarcoma accounts for one-fourth of the primary tumors in the head and neck region. Modern treatment modalities have led to a 2-year survival rate of about 90% in these patients. However, new therapeutic trials are designed to reduce complications and salvage more than 90% of orbital cases. Between 1979 and 1990, 12 children affected by primary orbital rhabdomyosarcoma have been diagnosed and treated at the University of Naples. Ten of them have been uniformly treated by biopsy, followed by immediate radiation and combined chemotherapy. All 12 patients are alive and free of detectable disease, from a minimum of 7 months to a maximum of 123 months after diagnosis. In all children, ocular structures have been spared and the complications observed until now have been few. The above results suggest that the association of immediate radiation therapy and chemotherapy might represent an optimal tool for treatment of orbital rhabdomyosarcoma.

[Cryptococcus meningitis: clinical course, development and histopathologic aspects depending on the predisposing factors]. / Meningite criptocócica: aspectos clínicos, evolutivos e histopatológicos segundo a condição predisponente.

Seventeen consecutive cases of cryptococcal meningitis diagnosed at Hospital das Clínicas of Ribeirão Preto Medical School (São Paulo State Brazil) between 1969 and 1985 were reviewed. For analysis the patients were separated in 3 groups: I. three patients without immunodeficiency; II. six patients with associated disease: cancer (3), diabetes (2) and alcoholism (1); III. Eight renal transplant recipients that developed cryptococcosis after 18 to 67 months of immunosupression with steroids and azathioprine. The median interval between onset of symptoms and diagnosis of infection was greater in Group II (53 days) than in Groups I (25 days) or III (28 days). Neck stiffness, cranial nerve involvement and papilledema were more frequent in Group I than in Group II or III, but fever and focal neurological signs were observed only in patients of two last groups. Cerebrospinal fluid examination showed a mild lymphocytic pleocytosis in most patients, but transplant cases had polymorphonuclear cells more frequently. Late mortality was higher in patients with underlying disease and the prognosis was better for transplant patients that received effective antifungal therapy. Besides cryptococci, autopsy findings in 8 cases revealed granuloma formation in tissues, except in patients of Group II (2 cases). The differences between the groups suggest that clinical characteristics, evolution and postmortem findings of the cryptococcal meningitis are changed according to type of immunodeficiency presented by the patient.

Eruptive familial angiolipomas occurring during pregnancy.

Multiple angiolipomas presenting acutely in a pregnant woman are described. To the best of our knowledge, onset of this eruption during pregnancy has not been reported previously.