Rare Childhood Cancers

and Daniela Cristina Stefan2



(1)
Université Mohammed VI des Sciences de la Santé Cheikh Khalifa Hospital, Casablanca, Morocco

(2)
South African Medical Research Council, Cape Town, South Africa

 



Keywords
Nasopharyngeal carcinomaJuvenile nasopharyngeal angiofibromaCarcinoid tumorsColorectal carcinomaPneublastomaGastrointestinal stromal tumors (GIST)Skin tumorsThymomaChordomaThyroid carcinomaMelanomaPheochromocytoma


This is a heterogeneous group of childhood cancers which can include low incidence tumors in human or adult tumors occurring in children. These tumors are mostly non-embryonic. Their incidence is higher among adolescents. The experience of a pediatrician may be limited and contribution of adult oncologist could prove beneficial.

In order to improve the knowledge about some of these tumors, data are collected in national or international registries.


Head and Neck Tumors



Nasopharyngeal Carcinoma


Carcinoma or nasopharyngeal lymphoepithelioma is a tumor that develops from the epithelium of the nasopharynx. These tumors have a high incidence in China, Southeast Asia, Alaska, and North Africa. They are extremely rare in the Southern part of the continent. This geographical distribution has led to incrimination to lifestyle-related risk factors including consumption of salted fish and meat. Predisposition is also reported in subjects with a HLA2 haplotype and HLA-B sin2 and AW19, BWA6, and B17. Furthermore the role of the Epstein Barr virus (EBV) is strongly suspected in the pathogenesis of this tumor. Patients with this type of cancer have high IgG and IgA directed against early EBV Ag or viral capsid. The viral genome is found at the level of malignant epithelial cells but not at the level of the infiltrating lymphocytes. Tumor cells express different genes of the EBV including EBNA1, LMP1, and EBER1.

Clinically, the tumor occurs usually in adolescence as a non-painful high cervical mass associated with cervical lymph nodes. The tumor may induce hearing difficulties, otitis, nasal obstruction, and epistaxis. The locoregional extension can infiltrate XIIth cranial nerve giving inducing dysphonia, or of VIth cranial nerve giving rise to diplopia. Unusually these tumors can be accompanied by a paraneoplastic syndrome (hypertrophic ostearthropathy, dematomyositis).

Pediatric forms are often extensive. Metastases may occur at the level of the lungs, bones, and bone marrow. The complete assessment includes an MRI. In absence of an MRI CT-scan contributes to the diagnosis. Distance metastases are ruled out by Chest radiography, abdominal ultrasound, CT-Scan, and bone scan. In case of skull base tumor infiltration, a cerebrospinal fluid analysis is indicated. Histopathology of nasopharyngeal carcinoma distinguishes type-I features or keratinizing squamocellular carcinoma often associated with alcohol and tobacco consumption but not the EBV type-II or squamous nonkeratinizing and type-III or lymphoepithelioma or undifferentiated carcinoma. Pediatric forms are almost exclusively of type-III.

Chemotherapy and radiotherapy are efficient in curing the disease.

Chemotherapy is recommended as the first-line treatment to reduce the size of the tumor and in this way also to reduce the dose and targeted volume of radiotherapy. The most used protocols combine cis-platinum, methotrexate, 5-fluorouracil paclitaxel and the carboplatin.


Esthesioneuroblastoma


This tumor originates from the olfactory nerve and has usually an intracranial extension. Clinical expression is usually anosmia, epistaxis, nasal obstruction, and cervical lymphadenopathies. The bone involvement with ethmoid and maxillary sinuses infiltration is commonly seen on CT-scan and/or MR.

The treatment combines surgery and irradiation. In extended forms, prolonged chemotherapy has shown a good and sometimes a complete response. It is however a rare tumor and not commonly known.


Juvenile Nasopharyngeal Angiofibroma


This is a malignant vascular tumor of the lateral wall of the nasopharynx with an extension to the adjacent structures including the sphenoid, orbital, maxillary, and the infratemporal fossa. It is found more frequently in patients suffering from a familial adenomatous polyposis. Clinical manifestations are characterized by epistaxis, cheek swelling, orbital tumor, and sometimes paralysis of cranial pairs. Surgical resection is recommended in localized forms. Irradiation with or without surgery may be necessary.


Carcinoid Tumors


These tumors of epithelial origin can be benign or malignant and are mainly at the appendix. They are more frequent in girls and sometimes are found incidentally in the pathology of appendectomy for acute appendicitis. They derive from chromaffin cells and are capable of producing vasoactive peptides (5-hydroxytryptamine).

Clinical expression may be vasomotor disorders, diarrhea, and bronchoconstriction. Surgical resection is the treatment of choice for these patients.


Colorectal Carcinoma


It is one of the most common cancers in adults. Less than 1% of these cancers occur in the population of less than 20 years of age. In children the tumor is most of the time undifferentiated. Family polyposis, Gardner syndrome, Turcot, and other polyposis syndromes increase the risk of colorectal carcinoma.

Clinical expression is variable according to the tumor site and is associated frequently with abdominal pain, anorexia, anemia, constipation or diarrhea with hemorrhage or melena. It can also present with gastrointestinal perforation or intestinal obstruction. The diagnosis is facilitated by exploration which includes barium enema, CT-scan or colonoscopy to characterize the tumor and search for possible metastases. A High level of carcinoembryonic antigen is associated with a poor prognosis.

Surgery is the main treatment of colorectal carcinoma. In the rectosigmoidal locations treatment combining 5 Fluorouracil-based chemotherapy and irradiation can be used to make the tumor operable.


Gastrointestinal Stromal Tumors (GIST)


These tumors occur also mainly in adults. They derive from primitive mesenchymal cells having developed a proto-oncogene c-kit mutation. They are located in more than half of the cases at the level of the stomach but can also interest other digestive segments or be multifocal. Abdominal pain, bleeding, perforation or a palpable mass are the main presenting features. Surgical removal of the tumor when possible is the treatment of choice. Tyrosine kinase inhibitors have also proved their efficiency in this type of cancer.

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Jun 26, 2017 | Posted by in PEDIATRICS | Comments Off on Rare Childhood Cancers

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