Use of DOTATATE PET/CT scan in the diagnosis and staging of thymic atypical carcinoid tumor in a patient with secondary ACTH-dependent cushing syndrome: Look twice and cut once

John Agzarian, Hisham Quandeel, Irina Bancos, Geoffrey B Johnson, Stephen C. Scharf, Geoffrey B. Thompson, Joanne Yi, Xiaotun Zhang, K. Robert Shen

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Neuroendocrine thymic tumors represent the least common type of primary thymic tumor with a prevalence of 2 to 5%. We present a case of locally advanced thymic atypical carcinoid tumor diagnosed incidentally while investigating progressive Cushing syndrome. Computed tomography (CT) scan demonstrated a large 2.9 cm exophytic thyroid nodule and a 2.0 cm anterior mediastinal mass. Biopsy of the thyroid nodule demonstrated benign thyroid tissue, and octreotide scan revealed avid uptake in the right thyroid lobe with minimal uptake in the thymic tumor.68Gallium-1,4,7,10-tetraazacyclododecane-N,N′,N″N′″-tetraacetic acid-D-Phe1,Tyr3-octreotate (Ga-68 DOTATATE) positron emission tomography (PET)/CT scan showed intense uptake in the thyroid gland followed by a moderate amount of activity in the anterior mediastinal mass. The patient underwent a median sternotomy and radical thymectomy with en bloc resection of the left innominate vein and primary repair of the superior vena cava (SVC). On further evaluation, the presumed thyroid nodule appeared contiguous with the thymic lesion and separate from the thyroid. Final pathology demonstrated a poorly differentiated 4.4 cm atypical carcinoid tumor with nine positive lymph nodes. On the third postoperative day, the serum cortisol level precipitously decreased to a level of 6.8 µg/dL. This case highlights the challenges surrounding the diagnosis of hormonally active thymic carcinoids and demonstrates the utility of Ga-68 DOTATATE PET scan in localizing somatostatin-positive neuroendocrine tumors and surgical planning.

Original languageEnglish (US)
Pages (from-to)127-133
Number of pages7
JournalWorld Journal of Endocrine Surgery
Volume10
Issue number2
DOIs
StatePublished - May 1 2018

Fingerprint

Cushing Syndrome
Carcinoid Tumor
Adrenocorticotropic Hormone
Thyroid Nodule
Thyroid Gland
Thymus Neoplasms
Neuroendocrine Tumors
Brachiocephalic Veins
Thymectomy
Gallium
Superior Vena Cava
Sternotomy
Octreotide
Somatostatin
Positron-Emission Tomography
Hydrocortisone
Lymph Nodes
Tomography
Pathology
Biopsy

Keywords

  • DOTATATE
  • Mediastinal
  • Positron emission tomography
  • Thymic carcinoid

ASJC Scopus subject areas

  • Surgery
  • Endocrinology, Diabetes and Metabolism
  • Radiology Nuclear Medicine and imaging

Cite this

Use of DOTATATE PET/CT scan in the diagnosis and staging of thymic atypical carcinoid tumor in a patient with secondary ACTH-dependent cushing syndrome : Look twice and cut once. / Agzarian, John; Quandeel, Hisham; Bancos, Irina; Johnson, Geoffrey B; Scharf, Stephen C.; Thompson, Geoffrey B.; Yi, Joanne; Zhang, Xiaotun; Shen, K. Robert.

In: World Journal of Endocrine Surgery, Vol. 10, No. 2, 01.05.2018, p. 127-133.

Research output: Contribution to journalArticle

Agzarian, John ; Quandeel, Hisham ; Bancos, Irina ; Johnson, Geoffrey B ; Scharf, Stephen C. ; Thompson, Geoffrey B. ; Yi, Joanne ; Zhang, Xiaotun ; Shen, K. Robert. / Use of DOTATATE PET/CT scan in the diagnosis and staging of thymic atypical carcinoid tumor in a patient with secondary ACTH-dependent cushing syndrome : Look twice and cut once. In: World Journal of Endocrine Surgery. 2018 ; Vol. 10, No. 2. pp. 127-133.
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abstract = "Neuroendocrine thymic tumors represent the least common type of primary thymic tumor with a prevalence of 2 to 5{\%}. We present a case of locally advanced thymic atypical carcinoid tumor diagnosed incidentally while investigating progressive Cushing syndrome. Computed tomography (CT) scan demonstrated a large 2.9 cm exophytic thyroid nodule and a 2.0 cm anterior mediastinal mass. Biopsy of the thyroid nodule demonstrated benign thyroid tissue, and octreotide scan revealed avid uptake in the right thyroid lobe with minimal uptake in the thymic tumor.68Gallium-1,4,7,10-tetraazacyclododecane-N,N′,N″N′″-tetraacetic acid-D-Phe1,Tyr3-octreotate (Ga-68 DOTATATE) positron emission tomography (PET)/CT scan showed intense uptake in the thyroid gland followed by a moderate amount of activity in the anterior mediastinal mass. The patient underwent a median sternotomy and radical thymectomy with en bloc resection of the left innominate vein and primary repair of the superior vena cava (SVC). On further evaluation, the presumed thyroid nodule appeared contiguous with the thymic lesion and separate from the thyroid. Final pathology demonstrated a poorly differentiated 4.4 cm atypical carcinoid tumor with nine positive lymph nodes. On the third postoperative day, the serum cortisol level precipitously decreased to a level of 6.8 µg/dL. This case highlights the challenges surrounding the diagnosis of hormonally active thymic carcinoids and demonstrates the utility of Ga-68 DOTATATE PET scan in localizing somatostatin-positive neuroendocrine tumors and surgical planning.",
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T2 - Look twice and cut once

AU - Agzarian, John

AU - Quandeel, Hisham

AU - Bancos, Irina

AU - Johnson, Geoffrey B

AU - Scharf, Stephen C.

AU - Thompson, Geoffrey B.

AU - Yi, Joanne

AU - Zhang, Xiaotun

AU - Shen, K. Robert

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N2 - Neuroendocrine thymic tumors represent the least common type of primary thymic tumor with a prevalence of 2 to 5%. We present a case of locally advanced thymic atypical carcinoid tumor diagnosed incidentally while investigating progressive Cushing syndrome. Computed tomography (CT) scan demonstrated a large 2.9 cm exophytic thyroid nodule and a 2.0 cm anterior mediastinal mass. Biopsy of the thyroid nodule demonstrated benign thyroid tissue, and octreotide scan revealed avid uptake in the right thyroid lobe with minimal uptake in the thymic tumor.68Gallium-1,4,7,10-tetraazacyclododecane-N,N′,N″N′″-tetraacetic acid-D-Phe1,Tyr3-octreotate (Ga-68 DOTATATE) positron emission tomography (PET)/CT scan showed intense uptake in the thyroid gland followed by a moderate amount of activity in the anterior mediastinal mass. The patient underwent a median sternotomy and radical thymectomy with en bloc resection of the left innominate vein and primary repair of the superior vena cava (SVC). On further evaluation, the presumed thyroid nodule appeared contiguous with the thymic lesion and separate from the thyroid. Final pathology demonstrated a poorly differentiated 4.4 cm atypical carcinoid tumor with nine positive lymph nodes. On the third postoperative day, the serum cortisol level precipitously decreased to a level of 6.8 µg/dL. This case highlights the challenges surrounding the diagnosis of hormonally active thymic carcinoids and demonstrates the utility of Ga-68 DOTATATE PET scan in localizing somatostatin-positive neuroendocrine tumors and surgical planning.

AB - Neuroendocrine thymic tumors represent the least common type of primary thymic tumor with a prevalence of 2 to 5%. We present a case of locally advanced thymic atypical carcinoid tumor diagnosed incidentally while investigating progressive Cushing syndrome. Computed tomography (CT) scan demonstrated a large 2.9 cm exophytic thyroid nodule and a 2.0 cm anterior mediastinal mass. Biopsy of the thyroid nodule demonstrated benign thyroid tissue, and octreotide scan revealed avid uptake in the right thyroid lobe with minimal uptake in the thymic tumor.68Gallium-1,4,7,10-tetraazacyclododecane-N,N′,N″N′″-tetraacetic acid-D-Phe1,Tyr3-octreotate (Ga-68 DOTATATE) positron emission tomography (PET)/CT scan showed intense uptake in the thyroid gland followed by a moderate amount of activity in the anterior mediastinal mass. The patient underwent a median sternotomy and radical thymectomy with en bloc resection of the left innominate vein and primary repair of the superior vena cava (SVC). On further evaluation, the presumed thyroid nodule appeared contiguous with the thymic lesion and separate from the thyroid. Final pathology demonstrated a poorly differentiated 4.4 cm atypical carcinoid tumor with nine positive lymph nodes. On the third postoperative day, the serum cortisol level precipitously decreased to a level of 6.8 µg/dL. This case highlights the challenges surrounding the diagnosis of hormonally active thymic carcinoids and demonstrates the utility of Ga-68 DOTATATE PET scan in localizing somatostatin-positive neuroendocrine tumors and surgical planning.

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KW - Positron emission tomography

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