Neuroendocrine tumor of thymus with ectopic production of adrenocorticotropic hormone in a 13-year-old child. Case report, 5-year follow-up

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Abstract

Neuroendocrine tumors (NET) of the thymus are rare, usually aggressive, and prone to metastatic lesion. Ectopic adrenocorticotropic hormone (ACTH) secretion in thymic NET (TNEN) is associated with poor prognosis. Most of TNET express somatostatin receptors, it allows the use of somatostatin receptor scintigraphy (SRS) and SPECT/CT or PET/CT with 68Ga-labeled peptides for diagnosis and staging of the disease. Surgery (macroscopic-complete resection) is the mainstay treatment for TNET. Now, there are no unequivocal data in adjuvant therapy and its effectiveness. We present a case report of the pediatric patient with well differentiated atypical ACTH-producing thymic carcinoid. This localization was verified by whole body somatostatin receptor scintigraphy with 111In-DTPA-octreotide (Octreoscan). The patient proceeded with the surgical treatment followed by remission of hypercorticism without adjuvant chemotherapy. Tumor recurrence with redevelopment of ACTH-ectopic syndrome was detected after 67 months of observation.

About the authors

Konstantin Iu. Slashchuk

Endocrinology Research Center

Email: slashuk911@gmail.com
ORCID iD: 0000-0002-3220-2438
SPIN-code: 3079-8033

Cand. Sci. (Med.)

Russian Federation, Moscow

Maria V. Reinberg

Endocrinology Research Center

Email: e.bazarova.bq@gmail.com
ORCID iD: 0009-0002-1632-2197

еndocrinologis

Russian Federation, Moscow

Ekaterina A. Bazarova

Endocrinology Research Center

Author for correspondence.
Email: e.bazarova.bq@gmail.com
ORCID iD: 0009-0009-7608-4726

endocrinologist

Russian Federation, Moscow

Maria A. Kareva

Endocrinology Research Center

Email: i_marusya@mail.ru
ORCID iD: 0000-0003-1320-6561
SPIN-code: 5089-0310

D. Sci. (Med.)

Russian Federation, Moscow

Elena G. Przhiyalkovskaya

Endocrinology Research Center

Email: e.bazarova.bq@gmail.com
ORCID iD: 0000-0001-9119-2447
SPIN-code: 9309-3256

Cand. Sci. (Med.), Assoc. Prof.

Russian Federation, Moscow

Mikhail V. Degtiarev

Endocrinology Research Center

Email: germed@mail.ru
ORCID iD: 0000-0001-5652-2607
SPIN-code: 7725-7831

Department Head

Russian Federation, Moscow

Oleg V. Pikin

Hertsen Moscow Oncology Research Institute – branch of the National Medical Research Radiological Centre

Email: e.bazarova.bq@gmail.com
ORCID iD: 0000-0001-6871-6804
SPIN-code: 2381-5969

D. Sci. (Med.)

Russian Federation, Moscow

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2. Fig. 1. Somatostatin receptor scintigraphy (SRS) and single-photon emission computed tomography with X-ray computed tomography with 111In-DTPA-octreotide (Octreoscan). Thymus mass with signs of increased radiopharmaceutical uptake (arrow).

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3. Fig. 2. PET/CT with 68Ga-DOTA-TATE. Recurrence of a neuroendocrine tumor of the thymus; a mass with signs of increased radiopharmaceutical uptake (arrow). One of the lesions (b) has no signs of increased radiopharmaceutical uptake (arrow).

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