Primary intracranial neuroendocrine tumor with ectopic adrenocorticotropic hormone syndrome: A rare and complicated case report and literature review

Liu,Hailong; Zhang,Mingshan; Wang,Xuan; Qu,Yanming; Zhang,Hongwei; Yu,Chunjiang

doi:10.3892/mco.2016.904

July-2016 Volume 5 Issue 1

Full Size Image

Journals

International Journal of Molecular Medicine

International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

International Journal of Oncology

International Journal of Oncology is an international journal devoted to oncology research and cancer treatment.

Molecular Medicine Reports

Covers molecular medicine topics such as pharmacology, pathology, genetics, neuroscience, infectious diseases, molecular cardiology, and molecular surgery.

Oncology Reports

Oncology Reports is an international journal devoted to fundamental and applied research in Oncology.

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine is an international journal devoted to laboratory and clinical medicine.

Oncology Letters

Oncology Letters is an international journal devoted to Experimental and Clinical Oncology.

Biomedical Reports

Explores a wide range of biological and medical fields, including pharmacology, genetics, microbiology, neuroscience, and molecular cardiology.

Molecular and Clinical Oncology

International journal addressing all aspects of oncology research, from tumorigenesis and oncogenes to chemotherapy and metastasis.

World Academy of Sciences Journal

Multidisciplinary open-access journal spanning biochemistry, genetics, neuroscience, environmental health, and synthetic biology.

International Journal of Functional Nutrition

Open-access journal combining biochemistry, pharmacology, immunology, and genetics to advance health through functional nutrition.

International Journal of Epigenetics

Publishes open-access research on using epigenetics to advance understanding and treatment of human disease.

Medicine International

An International Open Access Journal Devoted to General Medicine.

July-2016 Volume 5 Issue 1

Full Size Image

Case Report

Primary intracranial neuroendocrine tumor with ectopic adrenocorticotropic hormone syndrome: A rare and complicated case report and literature review

Authors:
- Hailong Liu
- Mingshan Zhang
- Xuan Wang
- Yanming Qu
- Hongwei Zhang
- Chunjiang Yu
View Affiliations / Copyright

Affiliations: Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, P.R. China
Pages: 99-102
|
Published online on: May 11, 2016

https://doi.org/10.3892/mco.2016.904
Expand metrics +

Abstract

Neuroendocrine tumors (NETs) and ectopic adrenocorticotropic hormone (ACTH) syndrome are frequent in adult patients. However, primary intracranial NETs, exhibiting immunonegativity for ACTH, high serum ACTH level and treated with anterior skull base reconstruction, are rare and complicated. We herein present a case of a primary intracranial NET immunonegative for ACTH, resulting in ectopic ACTH syndrome. A 40‑year‑old woman presented with intermittent rhinorrhea, rapid weight gain, polydipsia, polyuria, hypertension, dimness, bilateral exophthalmus, diminution of vision in the left eye and pigmentation of the skin of the face and trunk. Computed tomography (CT) and magnetic resonance imaging scans revealed a sizeable enhancing tumor in the anterior cranial fossa, which infiltrated the sphenoid and ethmoid sinuses bilaterally, the left maxillary sinus and the nasal cavity. Abdominal CT scans revealed bilateral adrenal hyperplasia. The biochemical findings included hypokalemia and high glucose, cortisol, plasma ACTH, 24‑h urinary free cortisol and testosterone levels. The neoplasm was exposed through a right frontal craniotomy, while anterior skull base reconstruction was performed during surgery. The intracranial surgery achieved gross removal of the tumor; however, part of the tumor remained in the nasal cavity. Histopathological examination of the surgical specimen confirmed the diagnosis of a low‑grade small‑cell NET, exhibiting immunonegativity for ACTH. A postoperative abdominal CT scan demonstrated bilateral regression of the adrenal gland hyperplasia and the serum ACTH level returned to normal after 16 days. To the best of our knowledge, there are no previous reports of primary intracranial NETs, immunohistochemically negative for ACTH, resulting in ectopic ACTH syndrome.

View Figures

View References

1	Godwin JD II: Carcinoid tumors. A analysis of 2837 cases. Cancer. 36:560–569. 1975. View Article : Google Scholar : PubMed/NCBI
2	Volante M, Rindi G and Papotti M: The grey zone between pure (neuro)endocrine and non-(neuro)endocrine tumors: A comment on concepts and classification of mixed exocrine-endocrine neoplasms. Virchows Arch. 449:499–506. 2006. View Article : Google Scholar : PubMed/NCBI
3	Shi J, Xu Z, Wang ZD, et al: Pathological category and diagnosis of neuroendocrine tumor in non-neuroendocrine system. J Clin Exp Pathol. 25:548–550. 2009.(In Chinese).
4	Faggiano A, Mansueto G, Ferolla P, Milone F, del Basso de Caro ML, Lombardi G, Colao A and De Rosa G: Diagnosis and prognostic implication of the World Health Organization classification of neuroendocrine tumors. J Endocrinol Invest. 31:216–223. 2008. View Article : Google Scholar : PubMed/NCBI
5	Lambert SW, Hofland LJ and Nobels FR: Neuroendocrine tumor markers. Front Neuroendocrinol. 22:309–339. 2001. View Article : Google Scholar : PubMed/NCBI
6	Klimstra DS: Pathology reporting of neuroendocrine tumors: Essential elements for accurate diagnosis, classification and staging. Semin Oncol. 40:23–36. 2013. View Article : Google Scholar : PubMed/NCBI
7	Travis WD, Rush W, Flieder DB, Falk R, Fleming MV, Gal AA and Koss MN: Survival analysis of 200 pulmonary neuroendocrine tumors with classification of criteria for atypical carcinoid and its separation from typical carcinoid. Am J Surg Pathol. 22:934–944. 1998. View Article : Google Scholar : PubMed/NCBI
8	Hochwald SN, Zee S, Conlon KC, Colleoni R, Louie O, Brennan MF and Klimstra DS: Prognostic factors in pancreatic endocrine neoplasms: An analysis of 136 cases with a proposal for low-grade and intermediate-grade groups. J Clin Oncol. 20:2633–2642. 2002. View Article : Google Scholar : PubMed/NCBI
9	Smith EM, Galin FS, LeBoeuf RD, Coppenhaver DH, Harbour DV and Blalock JE: Nucleotide and amino acid sequence of lymphocyte-derived corticotropin: Endotoxin induction of a truncated peptide. Pro Natl Sci USA. 87:1057–1064. 1990. View Article : Google Scholar
10	Kolesnikova GS, Lapshina AM, Voronkova IA, Marova EI, Arapova SD, Goncharov NP and Dedov II: Comparative analysis of clinical, hormonal and morphological studies in patients with neuroendocrine ACTH-producing tumors. Int J Endocrinol. 2013:6592322013. View Article : Google Scholar : PubMed/NCBI
11	Al-Gahtany M, Horvath E and Kovacs K: Pituitary hyperplasia. Hormones (Athens). 2:149–158. 2003. View Article : Google Scholar : PubMed/NCBI
12	Liu H, Zhang Q and Yang Z: Skull base reconstruction and rehabilitation. J Clin Otorhinolaryngol. 18:755–757. 2003.(In Chinese).

Journals

International Journal of Molecular Medicine

International Journal of Oncology

Molecular Medicine Reports

Oncology Reports

Experimental and Therapeutic Medicine

Oncology Letters

Biomedical Reports

Molecular and Clinical Oncology

World Academy of Sciences Journal

International Journal of Functional Nutrition

International Journal of Epigenetics

Medicine International

Primary intracranial neuroendocrine tumor with ectopic adrenocorticotropic hormone syndrome: A rare and complicated case report and literature review

This article is mentioned in:

Abstract

Figure 1

Figure 2

Figure 3

Figure 4

Figure 5

Figure 6

Figure 7

Related Articles