Review Paraneoplastic syndromes in olfactory neuroblastoma

Kunc, Michał; Gabrych, Anna; Czapiewski, Piotr; Sworczak, Krzysztof

doi:10.5114/wo.2015.46283

Cited by 24 publications

(36 citation statements)

References 87 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…A subset of ONB expresses somatostatin receptors (SSTRs), thus the octreotide radionuclide scans have been used during the diagnostic process, including ACTH-secreting ONB [75]. A recent study on ONB with meningeal metastases has displayed overexpression of SSTRs, which was documented by PET/CT after the administration of a 68 Gallium labeled somatostatin analog.…”

Section: Potential Targetable Pathwaysmentioning

confidence: 99%

Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatment

2016

Self Cite

View full text Add to dashboard Cite

Olfactory neuroblastoma (ONB, Esthesioneuroblastoma) is an infrequent neoplasm of the head and neck area derived from olfactory neuroepithelium. Despite relatively good prognosis a subset of patients shows recurrence, progression and/or metastatic disease, which requires additional treatment. However, neither prognostic nor predictive factors are well specified. Thus, we performed a literature search for the currently available data on disturbances in molecular pathways, cytogenetic changes and results gained by next generation sequencing (NGS) approaches in ONB in order to gain an overview of genetic alterations which might be useful for treating patients with ONB. We present briefly ONB molecular pathogenesis and propose potential therapeutic targets and prognostic factors. Possible therapeutic targets in ONB include: receptor tyrosine kinases (c-kit, PDGFR-b, TrkB; EGFR); somatostatin receptor; FGF-FGFR1 signaling; Sonic hedgehog pathway; apoptosis-related pathways (Bcl-2, TRAIL) and neoangiogenesis (VEGF; KDR). Furthermore, we compare high- and low-grade ONB, and describe its frequent mimicker: sinonasal neuroendocrine carcinoma. ONB is often a therapeutic challenge, so our goal should be the implementation of acquired knowledge into clinical practice, especially at pretreated, recurrent and metastatic stages. Moreover, the multicenter molecular studies are needed to increase the amount of available data.

show abstract

Section: Potential Targetable Pathwaysmentioning

confidence: 99%

Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatment

2016

Self Cite

View full text Add to dashboard Cite

show abstract

“…Further, in many patients the origin of malignancy is unknown (even up to 20%; the most commonly finally diagnosed tumour is a bronchial NEN) [5,12,13]. Causes of EAS are mentioned in Table II [6,7,11,[14][15][16][17].…”

Section: Szkolenie Podyplomowementioning

confidence: 99%

“…Dodatkowo dużą grupę stanowią pacjenci z nieznanym punktem wyjścia choroby nowotworowej, których odsetek w zależności od piśmiennictwa ocenia się na 20% przypadków (najczęściej ostateczne rozpoznanie to NEN oskrzeli) [5,12,13]. Wszystkie możliwe przyczyny EAS przedstawiono w tabeli II [6,7,11,[14][15][16][17].…”

Section: Epidemiologiaunclassified

Zespół Cushinga w przebiegu ektopowego wydzielania ACTH

Cieszyński

Berendt-Obołończyk

Szulc

et al. 2015

Endokrynologia Polska

Self Cite

View full text Add to dashboard Cite

Cushing's syndrome (CS) is defined as a constellation of clinical signs and symptoms occurring due to hypercortisolism. Cortisol excess may be endogenous or exogenous. The most common cause of CS is glucocorticoid therapy with supraphysiological (higher than in the case of substitution) doses used in various diseases (e.g. autoimmune). One possible CS cause is ectopic (extra-pituitary) ACTH secretion (EAS) by benign or malignant tumours. Since its first description in 1963, EAS aetiology has changed, i.e. as well as small cell lung cancer (SCLC), higher incidence in other malignancies has been reported. Ectopic ACTH secretion symptoms are usually similar to hypercortisolism symptoms due to other causes. A clinical suspicion of CS requires laboratory investigations. There is no single and specific laboratory test for making a CS diagnosis, and therefore multiple dynamic tests should be ordered. A combination of multiple laboratory noninvasive and invasive tests gives 100% sensitivity and 98% specificity for EAS diagnosis. If the EAS is caused by localised malignancy, surgery is the optimal treatment choice. Radical tumour excision may be performed in 40% of patients, and 80% of them are cured of the disease. The authors present an interesting clinical case of EAS, which is always a huge diagnostic challenge for clinicians. StreszczenieZespół objawów klinicznych: podmiotowych i przedmiotowych, które wynikają z hiperkortyzolemii określa się mianem zespołu Cushinga (CS). Nadmiar kortyzolu w organizmie może mieć źródło egzo-lub endogenne. Powszechnie uważa się, że najczęstszą przyczyną CS jest terapia glikokortykosteroidem w dawkach farmakologicznych, większych niż substytucyjne, z powodu różnych chorób, na przykład tkanki łącznej. Jedną z możliwych przyczyn CS jest ektopowe, czyli poza przysadkowe wydzielanie ACTH, przez nowotwór łagodny lub złośliwy. Od czasu pierwszego opisu w 1963 roku, etiologia ektopowego wydzielania ACTH uległa istotnej zmianie, poza drobnokomórko-wym rakiem płuca (SCLC) opisano inne nowotwory, których częstość znacząco wzrosła. Symptomatologia CS w przebiegu ektopowego wydzielania ACTH najczęściej odpowiada objawom hiperkortyzolemii spotykanym u chorych z powodu innej przyczyny. Podejrzenie kliniczne CS wymaga weryfikacji laboratoryjnej. Nie ma swoistego, pojedynczego badania, które by potwierdziło CS w przebiegu ektopowego wydzielania ACTH, z tego względu należy posiłkować się szeregiem testów. Połączenie wielu badań laboratoryjnych, w tym inwazyjnych, sprawia, że czułość rozpoznania ektopowego wydzielania ACTH sięgnąć może 100%, przy swoistości 98%. W przypadku lokalizacji ogniska pierwotnego leczenie operacyjne jest postępowaniem optymalnym. Radykalne leczenie można zastosować u 40% chorych, z czego około 80% udaje się wyleczyć. Przedstawiony przypadek ektopowego wydzielania ACTH stanowi przykład licznych trudności diagnostycznych dla lekarza praktyka.

show abstract

“…ON is rare (accounting for 3 to 10% of all intranasal tumors), with a bimodal age distribution with peaks in the second and fifth decades (1). Initial localization concerns the nasal cavity, with extension to ethmoid sinus, anterior skull base, and orbit (2). It is a slowly growing tumor and diagnosed at advanced stages in most cases.…”

Section: Introductionmentioning

confidence: 99%

An Endocrine Picture In Disguise: A Progressive Olfactory Neuroblastoma Complicated With Ectopic Cushing Syndrome

Clotman

Twickler

Dirinck

et al. 2017

AACE Clinical Case Reports

View full text Add to dashboard Cite

Objective:To describe a unique case of a 61-yearold patient presenting with ectopic Cushing syndrome as a paraneoplastic phenomenon due to adrenocorticotropic hormone (ACTH)-secreting bone metastasis of an olfactory neuroblastoma (ON).Methods: We report a detailed case, with clinical, biochemical, and imaging findings and compare it with existing literature.Results: A patient with an ON, with subsequent 10-year stable disease after initial craniofacial surgery, presented with paraparesis due to progressively metastasized ON disease located in the thoracic spine. During palliative chemotherapy, severe hypokalemia and muscle weakness developed, being part of paraneoplastic Cushing syndrome. Examination showed that metastatic bone lesions were mostly responsible for the ectopic secretion of ACTH, with just a few ACTH-secreting tumor cells located in the primary tumor. She was treated with a combination of ketoconazole and metyrapone that controlled cortisol levels. However, low potassium levels persisted. Finally, she underwent a bilateral adrenal resection, after which most cushingoid symptoms disappeared. She died 1 month later. Conclusion:Our case shows a growing ON with several bone metastases, which is a rare clinical condition. Moreover, this is the first report that ON is associated with bone metastases that express excessive ACTH secretion (and suggesting dedifferentiation of the primary tumor during chemotherapy treatment). Ectopic Cushing syndrome (with ACTH secreted by ON bone metastasis) can present late in the clinical trajectory. Finally, a bilateral adrenalectomy was needed to correct the life-threatening hypokalemia as part of the ectopic Cushing syndrome. (AACE Clinical Case Rep. 2017;3:e278-e283) Abbreviations: ACTH = adrenocorticotropic hormone; CRH = corticotropin-releasing hormone; IPSS = inferior petrosal sinus smapling; ON = olfactory neuroblastoma

show abstract

Review Paraneoplastic syndromes in olfactory neuroblastoma

Cited by 24 publications

References 87 publications

Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatment

Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatment

Zespół Cushinga w przebiegu ektopowego wydzielania ACTH

An Endocrine Picture In Disguise: A Progressive Olfactory Neuroblastoma Complicated With Ectopic Cushing Syndrome

Contact Info

Product

Resources

About