Vasoactive Intestinal Peptide-Producing Neuroblastic Tumors: A Rare Cause of Refractory Diarrhea.

Neuroblastic tumors are the most common malignant extracranial solid tumors of childhood. A small subgroup presents chronic incoercible diarrhea due to the tumor's production of vasoactive intestinal peptide (VIP). The hypothesis of an occult tumor is not always considered, which delays and impairs treatment. We aim to identify these patients' characteristics and help alert health professionals to the hypothesis of a neuroblastic tumor in children with chronic diarrhea refractory to the usual approach. We carried out an epidemiological study on all retrievable reports of neuroblastic tumors between 1975 and 2021 described in the Medical Literature Analysis and Retrieval System Online (MEDLINE), Excerpta Medica database (EMBASE), and Latin American & Caribbean Health Sciences Literature (LILACS) databases. Patient information was divided into categories, and we performed a descriptive analysis. We analyzed 96 cases; 83 (86.5%) cases had diarrhea prior to the diagnosis of the neoplasm, 49 (51%) were ganglioneuroblastomas, 69 (71.8%) were abdominal, and 59 of the 60 patients (98%) with reported acid-base disorders had hypokalemia. When serum VIP was reported, the majority of values varied between one and 20 times the upper reference limit. Seventy-two (75%) patients underwent complete tumor resection, and the overall survival rate was 70%. Serum VIP production by neuroblastic tumors is related to cell differentiation and better prognosis. Such children often require intensive hospital support to reverse the malnutrition and acid-base disorders related to this paraneoplastic syndrome. Its early diagnosis and treatment significantly change the prognosis and quality of life. We, therefore, suggest screening for neuroblastic tumors when health professionals encounter unmanageable chronic secretory diarrhea in children with no defined etiology in the usual investigations.

Photodynamic therapy to control oral candidiasis in a pediatric patient undergoing head and neck radiotherapy.

Candida albicans is an opportunistic yeast of the oral microbiome which can cause candidiasis in systemically susceptible individuals, such as those undergoing cancer treatment. The local management of the oral lesions is challenging and may be significantly improved with photodynamic therapy (PDT). PDT consists of an association of light with a photosensitizing agent and oxygen, whose interaction produces reactive oxygen species capable of reducing non-specific microbial contamination without causing side effects. This study aimed to report a case in which oral candidiasis was managed with PDT. A 12-year-old male patient undergoing head and neck radiotherapy (HNRT) for Undifferentiated Mesenchymal Neoplasm presented with dysgeusia, oral pain, and yeast infection on the 13th session of HNRT. The patient used topical antifungals for 30 consecutive days with no lesion or symptom remission. PDT was then proposed and the lesion was healed after a single PDT session, with no further clinical signs of infection and return to normal oral function. Local and non-invasive management of oral candidiasis is of utmost importance for immunocompromised patients, with good acceptability, no systemic side effects, and no drug interactions.

Treatment of Pediatric Adrenocortical Carcinoma With Surgery, Retroperitoneal Lymph Node Dissection, and Chemotherapy: The Children's Oncology Group ARAR0332 Protocol.

PURPOSE: Adrenocortical carcinoma (ACC) is a rare aggressive pediatric malignancy with distinct biology. Its treatment follows the principles developed for adults; pediatric-specific studies are scarce. PATIENTS AND METHODS: Prospective single-arm risk-stratified interventional study. Study objectives were (1) to describe the outcome of patients with stage I ACC treated with adrenalectomy alone; (2) to describe the outcome of stage II patients (completely resected > 200 cc or > 100 g) treated with adrenalectomy and retroperitoneal lymph node dissection; and (3) to describe the outcome of patients with stage III or IV treated with mitotane and chemotherapy. RESULTS: Between September 2006 and May 2013, 78 patients (77 eligible, 51 females) were enrolled. The 5-year event-free survival estimates for stages I (24 patients), II (15 patients), III (24 patients), and IV (14 patients) were 86.2%, 53.3%, 81%, and 7.1%, respectively. The corresponding 5-year overall survival estimates were 95.2%, 78.8%, 94.7%, and 15.6%, respectively. On univariate analysis, age, stage, presence of virilization, Cushing syndrome, or hypertension, germline TP53 status, and presence of a somatic ATRX mutation were associated with outcome. On multivariable analysis, only stage and age were significantly associated with outcome. The probabilities of mitotane and chemotherapy feasibility events were 10.5% and 31.6%, respectively. CONCLUSION: Outcome for children with stage I ACC is excellent with surgery. Outcome for patients with stage II disease is inferior despite retroperitoneal lymph node dissection. Patients with stage III ACC have an excellent outcome combining surgery and chemotherapy. Patients with stage IV ACC are older and have a poor outcome; new treatments should be explored for this high-risk group. The combination of mitotane and chemotherapy as prescribed in ARAR0332 resulted in significant toxicity; one third of patients with advanced disease could not complete the scheduled treatment.

Gorlin-Goltz syndrome and neoplasms: a case study.

UNLABELLED: Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome. OBJECTIVE: To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.

Rhabdomyosarcoma of the oral tissues--two new cases and literature review.

Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. About 35% of RMS arises in the head and neck, being classified as parameningeal and non-orbital non-parameningeal forms. Parameningeal tumors carry the worst prognosis. The use of contemporary, multi-agent chemotherapy, radiotherapy, and surgery has made treatment of the disseminated disease possible, and has significantly improved overall survival from 25% in 1970 to 70% in 1991. Here, we present the management of two cases of orofacial RMS in adolescents: an 18-year-old, white female that had a 9-month history of a nodule in the left buccal mucosa, and a 19-year-old, white male who had been aware of a nodule in the left, posterior maxillary ridge with progressive growth for 4 months. Before final diagnosis, both cases were previously treated as inflammatory lesions. Their clinicopathological aspects, treatment, and poor survival as a consequence of delays in diagnosis are discussed.

Dental abnormalities in children submitted to antineoplastic therapy.

PURPOSE: The purpose of this study was to assess, by panoramic radiographs, the prevalence of morphological dental changes in children with cancer who were submitted for chemotherapy alone or concomitant radiotherapy of the head and neck. METHODS: All patients admitted between March, 1996 and February, 2004 were analyzed and 137 were included in this retrospective, nonrandomized, institutional study. The rates of microdontia, taurodontia, anodontia, macrodontia, blunt root, and tapered root were assessed. RESULTS: The patients were distributed into 2 groups: (1) those with lymphoproliferative neoplasias (61%); and (2) those with solid tumors (39%). Their mean age when treatment began was 5 years and 6 months. Dental abnormalities were found in 39 (29%) patients, while 98 (72%) patients did not present any abnormality. The abnormalities found were: (1) microdontia (7%; N= 10); (2) anodontia (6%; N=8); (3) taurodontia (14%; N=19); (4) macrodontia (5%; N=7); (5) blunted root (2%; N=2); and (6) tapered root (4%; N=5). Of these patients: 22% (N=30) presented 1 abnormality; 4% (N=6) presented 2 abnormalities; and 2% (N=3) presented 3 abnormalities. CONCLUSION: Taurodontia was the most frequent abnormality found in children and adolescents who underwent antineoplastic treatment, and its rate was significantly higher than those found for the healthy Brazilian population. This study's results show that it is necessary for the odontologist to systematically research the dental changes that occur among this special group of patients.

Testicular tumors in children and adolescents.

OBJECTIVE: To perform a comparative assessment of the clinical and epidemiological aspects of testicular tumors in childhood and adolescence. METHODS: Retrospective analysis of medical records of patients with testicular or paratesticular neoplasms. Patients under 10 years were classified as children and patients between 10 and 20 were classified as adolescents. The obtained results were compared through the two-sample test for proportions: non-parametric Mann-Whitney test and log-rank test. RESULTS: 60 patients were admitted in the period from January 1992 to July 2009: 34 children and 26 adolescents with testicular or paratesticular neoplasms. The main manifestations were testicular tumor and scrotal pain. Pain complaints were more common in adolescents (p = 0.006), who presented a mean time from disease onset to diagnosis of 4.9 months, longer than children, who presented a period of 2.3 months from disease onset to diagnosis (p = 0.01). Histological types were as follows: germ cell tumors in 32/60 (53%), rhabdomyosarcomas (RMSs) in 23/60 (38.3%), and other in 5/60 (8.3%). Adolescents presented a higher incidence of RMSs, lymph node metastases (p = 0.003) and distant metastases (p = 0.035). Differences in survival rates among the studied patients were not statistically significant, the only indicative being that survival in RMS cases is longer for children (p = 0.072). CONCLUSIONS: Compared to children, adolescents with testicular tumor presented longer time from disease onset to diagnosis, aggressive histological type and advanced illness at diagnosis, despite the small sample analyzed.

Dental maturity assessment in children with acute lymphoblastic leukemia after cancer therapy.

UNLABELLED: Dental age is largely used in both forensic studies and clinical practice. All over the world, many studies have been made to determine chronological age using dental ages of individuals, but selecting individuals with no chronic or acute sickness as the study group. Cancer is the second most frequent cause of death in children, and acute lymphoblastic leukemia (ALL) is the most common type of cancer in childhood. Most of the children who survived childhood cancer experienced disturbances in dental development due to cancer therapy or to cancer itself. The aim of this study is to assess dental development in children and teenagers who had suffered from childhood leukemia and were submitted to chemotherapy isolated or associated with radiotherapy, by comparing the dental ages with those corresponding features in a healthy control group. Dental development was analyzed using panoramic radiographs of 92 children divided in two groups: 46 children between 5 and 12 years old, treated for ALL at GRAACC-IOP, UNIFESP, and as a control group, 46 healthy children treated for dental reasons at APCD, São Paulo. The dental age of the subjects was estimated using the system of Demirjian et al. [A. Demirjian, H. Goldstein, J.M. Tanner, A new system of dental age assessment, Hum. Biol. 45(2) (1973) 211-227]. A significant difference was found between the chronological and dental age of patients submitted to antineoplasic therapy for ALL, when compared to those of the control group, but there were no significant differences between patients treated with different protocols for ALL. CONCLUSIONS: although the study was within a small group of patients, we could clearly conclude that antineoplastic therapy can interfere in the dental maturity of patients treated for childhood cancer by interfering in dental formation and root development.

Tumores de testículo em crianças e adolescentes / Testicular tumors in children and adolescents

OBJETIVO: Avaliar comparativamente aspectos clínicos e epidemiológicos dos tumores de testículo na infância e adolescência. MÉTODOS: Análise retrospectiva dos prontuários de pacientes com neoplasias de testículo ou paratesticulares. Foram classificados como crianças os menores de 10 anos e como adolescentes os pacientes entre 10 e 20 anos. Os resultados obtidos foram comparados por meio do teste para duas proporções: teste não paramétrico de Mann-Whitney e teste de log-rank. RESULTADOS: No período de janeiro de 1992 a julho de 2009, foram admitidos 60 pacientes: 34 crianças e 26 adolescentes com neoplasias de testículo ou paratesticulares. As principais manifestações foram tumor e dor na bolsa escrotal. A queixa de dor foi mais comum em adolescentes (p = 0,006). Estes apresentaram tempo médio de história de 4,9 meses, mais prolongado do que crianças, com 2,3 meses (p = 0,01). Os tipos histológicos encontrados foram: tumores de células germinativas em 32/60 (53 por cento), rabdomiossarcomas (RMSs) em 23/60 (38,3 por cento) e outros em 5/60 (8,3 por cento). Os adolescentes apresentaram maior frequência de RMSs, metástases em linfonodos (p = 0,003) e a distância (p = 0,035). As diferenças na sobrevida dos pacientes estudados não foram estatisticamente significantes, havendo apenas indicativo de que a sobrevida, nos casos de RMS, é maior nas crianças (p = 0,072). CONCLUSÕES: Os adolescentes com tumor testicular apresentaram maior tempo de história, tipo histológico agressivo e doença avançada ao diagnóstico quando comparados às crianças, a despeito da pequena amostra.

OBJECTIVE: To perform a comparative assessment of the clinical and epidemiological aspects of testicular tumors in childhood and adolescence. METHODS: Retrospective analysis of medical records of patients with testicular or paratesticular neoplasms. Patients under 10 years were classified as children and patients between 10 and 20 were classified as adolescents. The obtained results were compared through the two-sample test for proportions: non-parametric Mann-Whitney test and log-rank test. RESULTS: 60 patients were admitted in the period from January 1992 to July 2009: 34 children and 26 adolescents with testicular or paratesticular neoplasms. The main manifestations were testicular tumor and scrotal pain. Pain complaints were more common in adolescents (p = 0.006), who presented a mean time from disease onset to diagnosis of 4.9 months, longer than children, who presented a period of 2.3 months from disease onset to diagnosis (p = 0.01). Histological types were as follows: germ cell tumors in 32/60 (53 percent), rhabdomyosarcomas (RMSs) in 23/60 (38.3 percent), and other in 5/60 (8.3 percent). Adolescents presented a higher incidence of RMSs, lymph node metastases (p = 0.003) and distant metastases (p = 0.035). Differences in survival rates among the studied patients were not statistically significant, the only indicative being that survival in RMS cases is longer for children (p = 0.072). CONCLUSIONS: Compared to children, adolescents with testicular tumor presented longer time from disease onset to diagnosis, aggressive histological type and advanced illness at diagnosis, despite the small sample analyzed.

Câncer na infância / Cancers in childhood

A despeito da raridade do câncer na infância, ocorrem cerca de 12.400 casos novos de câncer por ano nos EUA em crianças e adolescentes com idade inferior a 20 anos. O diagnóstico precoce do câncer nesta faixa etária é usualmente difícil de ser realizado em estádios iniciais, pois os sinais e sintomas das neoplasias malignas são relativamente inespecíficos e mimetizam uma variedade de outras doenças frequentes na criança. O objetivo deste artigo é discutir a incidência dos tumores e os sinais e sintomas de alarme, que requerem imediata avaliação e estabelecimento do diagnóstico. Quando possível, a criança com suspeita de câncer e seus familiares devem ser encaminhados para centro de oncologia pediátrico. Embora a incidência de doença maligna na infância seja baixa, o impacto do diagnóstico do câncer torna imperativo que todos os profissionais da saúde inseridos na Pediatria tenham alto índice de suspeita de câncer. Diagnóstico precoce de câncer pode salvar a vida de uma criança...

Rhabdomyosarcoma of the Oral Tissues – two new cases and literature review

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Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. About 35% of RMS arises in the head and neck, being classified as parameningeal and non-orbital non-parameningeal forms. Parameningeal tumors carry the worst prognosis. The use of contemporary, multi-agent chemotherapy, radiotherapy, and surgery has made treatment of the disseminated disease possible, and has significantly improved overall survival from 25% in 1970 to 70% in 1991. Here, we present the management of two cases of orofacial RMS in adolescents: an 18-year-old, white female that had a 9-month history of a nodule in the left buccal mucosa, and a 19-year-old, white male who had been aware of a nodule in the left, posterior maxillary ridge with progressive growth for 4 months. Before final diagnosis, both cases were previously treated as inflammatory lesions. Their clinicopathological aspects, treatment, and poor survival as a consequence of delays in diagnosis are discussed

Rabdomiosarcoma de cabeça e pescoço na infância / Head and neck rhabdomyosarcoma in childhood

Rabdomiosarcoma é uma neoplasia maligna originária de células mesenquimais primitivas, podendo ocorrer em qualquer lugar do corpo. É o sarcoma de partes moles mais comum na infância, e localiza-se mais freqüentemente na cabeça e pescoço. OBJETIVO: Estudar a ocorrência de RMS na cabeça e pescoço na infância, correlacionando aspectos clínicos e histopatológicos. FORMA DE ESTUDO: Clínico retrospectivo. MÉTODO: Oitenta e dois pacientes com diagnóstico de sarcomas de partes moles, atendidos no Instituto de Oncopediatria da UNIFESP-EPM de 1988 a 2002 foram incluídos neste estudo. Foram estudados os seguintes parâmetros: incidência de RMS na infância e na cabeça e pescoço, distribuiçäo segundo sexo, faixa etária, tipo histológico, localizaçäo primária, óbito X localizaçäo, causa mortis. RESULTADOS: Neste estudo 65 por cento dos casos de sarcomas de partes moles corresponderam à RMS; 33 por cento dos casos de RMS localizavam-se na cabeça e pescoço; 77 por cento dos casos de sarcoma de partes moles de cabeça e pescoço corresponderam ao RMS. A média de idade no diagnóstico foi de 7,62 anos, predominando na faixa etária dos 5 aos 9 anos (41 por cento). Em relaçäo ao sexo, encontramos 47 por cento do sexo feminino e 53 por cento do sexo masculino. Quanto ao tipo histológico, o mais comum foi o RMS embrionário correspondendo a 64,6 por cento do total. O sítio primário mais comum foi o orbital (52,8 por cento). Cem por cento, 50 por cento e 33,3 por cento dos pacientes com RMS parameníngeo, näo parameníngeo e orbital, respectivamente, evoluíram para óbito. CONCLUSÄO: O RMS é o sarcoma de partes moles mais comum na infância, localizando-se preferencialmente na cabeça e pescoço. Houve predominância do sexo masculino neste estudo; idade média de 7,62 anos predominando a faixa etária dos 5 aos 9 anos. O tipo histológico predominante foi o embrionário e a localizaçäo orbital foi mais freqüente. O maior índice de óbito pertenceu aos RMS parameníngeo e o menor ao orbital

Tumor de células de Sertoli-Leydig do ovário: apresentação de um caso e revisão da literatura / Ovary tumor by Sertoli-Leydig cells tumor: a case report and review of the literature

Os autores relatam um caso de tumor de células de Sertoli e Leydig (TCSL), estádio Ia, no ovário direito de uma paciente com 10 anos de idade. O tumor era não funcionante e as manifestações iniciais foram dor e aumento do volume abdominal. Ao exame físico palpava-se uma massa endurecida na região hipogástrica. A criança não apresentava sinais de virilização ou puberdade precoce. O tratamento foi realizado com cirurgia: salpingo-ooforectomia direita e biópsia em cunha do ovário esquerdo. Histologicamente o tumor era bem diferenciado e não apresentava elementos heterólogos. A paciente apresenta há 26 meses sobrevida livre de doença. Acreditamos que este seja o primeiro caso de TCSL de ovário em crianças com idade igual ou inferior a 10 anos, publicado na literatura médica brasileira indexada.