Identification of Growth Hormone Receptor in Plexiform Neurofibromas of Patients with Neurofibromatosis Type 1

Cunha, Karin Soares Gonçalves; Barboza, Eliane Porto; Fonseca, Elize Massard da

doi:10.1590/s1807-59322008000100008

Cited by 25 publications

(22 citation statements)

References 29 publications

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“…Careful consideration must be taken when determining whether to initiate therapy by balancing the risk of prolonged exposure to elevated GH levels in tumors known to have GH receptors [112, 121] with the potentially transient nature of the GH excess [111, 122]. …”

Section: Treatment Considerationsmentioning

confidence: 99%

Growth Disturbances in Childhood Cancer Survivors

Antal

Balachandar

2019

Horm Res Paediatr

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Survival from childhood cancer has improved dramatically over the last few decades, resulting in an increased need to address the long-term follow-up and care of childhood cancer survivors. Appropriate linear growth is an important measure of health, with alterations of growth in children and short adult height in those who have completed growth serving as potential indicators of the sequelae of the underlying diagnosis or the cancer treatments. It is therefore critical that clinicians, particularly endocrinologists, be familiar with the patterns of altered growth which may be seen following diagnosis and treatment for childhood cancer. In this article, we will review the growth alterations seen in childhood cancer survivors, focusing on risk factors and considerations in evaluation and care.

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Section: Treatment Considerationsmentioning

confidence: 99%

Growth Disturbances in Childhood Cancer Survivors

Antal

Balachandar

2019

Horm Res Paediatr

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“…To date, no randomized trial of GH therapy in NF1 children has been completed. Recently, GHR expression was investigated in 5 plexiform neurofibromas from NF1 patients using immunohistochemistry [30]. Four of the 5 tumors were found immunopositive for GHR.…”

Section: Ghd In Nf1 Childrenmentioning

confidence: 99%

Endocrine Implications of Neurofibromatosis 1 in Childhood

Bizzarri

Bottaro

2015

Horm Res Paediatr

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In 1882, von Recklinghausen described a group of patients with multiple tumors arising from the ‘endoneurium' of peripheral nerves, and called them ‘neurofibromas'. The term von Recklinghausen disease was used up to the end of the 20th century, when the gene of neurofibromatosis (NF1) was cloned on chromosome 17q11.2. The gene product is a cytoplasmic protein termed neurofibromin, regulating proliferation and maturation of both glial and neuronal progenitors during embryogenesis. Loss of neurofibromin function determines the hyperactivation of the proto-oncogene RAS, leading to an increased risk of tumor formation, predominantly affecting the skin, bone and the nervous system. NF1 is clinically and genetically distinct from neurofibromatosis type 2, characterized by bilateral vestibular schwannomas and other nervous system tumors. An increased incidence of central precocious puberty, diencephalic syndrome, GH deficiency and GH hypersecretion has been described in NF1 children. These conditions are commonly complications of optic pathway gliomas (OPG) involving the hypothalamic and sellar region. Nevertheless, these endocrine disorders have been observed also in children without evidence of OPG at magnetic resonance imaging. Clinical and laboratory follow-up is crucial in all children with NF1, particularly in those with an OPG, aiming at the early identification of signs suggestive of secondary endocrine alterations.

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“…All the cases were used in previous studies (Cunha et al 2003(Cunha et al , 2008(Cunha et al , 2009. The samples of the tumors were included in two tissue microarray (TMA) paraffin blocks.…”

Section: Samplementioning

confidence: 99%

Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

Cunha

Caruso²,

Faria³

et al. 2013

An. Acad. Bras. Ciênc.

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AIMS:To study the expression of Bcl-2, Bcl-x, as well the presence of cleaved caspase-3 in neurofibromas and malignant peripheral nerve sheath tumors. The expression of Bcl-2 and Bcl-x and the presence of cleaved caspase 3 were compared to clinicopathological features of malignant peripheral nerve sheath tumors and their impact on survival rates were also investigated. MATERIALS AND METHODS: The evaluation of Bcl-2, Bcl-x and cleaved caspase-3 was performed by immunohistochemistry using tissue microarrays in 28 malignant peripheral nerve sheath tumors and 38 neurofibromas. Immunoquantification was performed by computerized digital image analysis. CONCLUSIONS: Apoptosis is altered in neurofibromas and mainly in malignant peripheral nerve sheath tumors. High levels of cleaved caspase-3 are more common in tumors with more aggressive histological features and it is associated with lower disease free survival of patients with malignant peripheral nerve sheath tumors.

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Identification of Growth Hormone Receptor in Plexiform Neurofibromas of Patients with Neurofibromatosis Type 1

Cited by 25 publications

References 29 publications

Growth Disturbances in Childhood Cancer Survivors

Growth Disturbances in Childhood Cancer Survivors

Endocrine Implications of Neurofibromatosis 1 in Childhood

Evaluation of Bcl-2, Bcl-x and Cleaved Caspase-3 in Malignant Peripheral Nerve Sheath Tumors and Neurofibromas

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