INTERESTING IMAGE
Pituitary Involvement of Langerhans Cell Histiocytosis in an Adult Unveiled by FDG PET/CT Huijun Ju, MD,* Yu Pan, MD,* Jing Lv, MD,* Xinwu Mao, MD,† and Yifan Zhang, MD, PhD* Abstract: A 39-year-old woman with complains of polydipsia and dieresis was admitted to endocrinology service for evaluation and potential treatment of diabetes insipidus. During the hospitalization, the patient was found to have unexplained fever, for which an FDG PET/CT was performed to explore the source. The images revealed multiple regions of abnormal hypermetabolism throughout the body. Notably, there was an intense activity in the region of pituitary stalk. Pathological results after biopsy showed pituitary involvement of Langerhans cell histiocytosis. Key Words: Langerhans cell histiocytosis, pituitary, FDG PET/CT (Clin Nucl Med 2015;40: 509–511)
Received for publication September 25, 2014; revision accepted October 27, 2014. From the Departments of *Nuclear Medicine, and †Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, People's Republic of China. Conflicts of interest and sources of funding: none declared. Reprints: Yifan Zhang, MD, PhD, Department of Nuclear Medicine Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No. 197, Rui Jin 2nd Rd, Shanghai 200025, People's Republic of China. E-mail: [email protected]. Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0363-9762/15/4006–0509
REFERENCES 1. Buch-Olsen KM, Andersen RV, Hess S, et al. 18F-FDG-PET/CT in fever of unknown origin: clinical value. Nucl Med Commun. 2014;35:955–960. 2. Kjaer A, Lebech AM, Eigtved A, et al. Fever of unknown origin: prospective comparison of diagnostic value of 18F-FDG PET and 111In-granulocyte scintigraphy. Eur J Nucl Med Mol Imaging. 2004;31:622–626. 3. Ma Y, Li F, Chen L. Widespread hypermetabolic lesions due to multicentric form of Castleman disease as the cause of fever of unknown origin revealed by FDG PET/CT. Clin Nucl Med. 2013;38:835–837. 4. Meller J, Sahlmann CO, Scheel AK. 18F-FDG PET and PET/CT in fever of unknown origin. J Nucl Med. 2007;48:35–45. 5. Solav SV. FDG PET/CT in evaluation of pyrexia of unknown origin. Clin Nucl Med. 2011;36:e81–e86. 6. Tokmak H, Ergonul O, Demirkol O, et al. Diagnostic contribution of (18)F-FDGPET/CT in fever of unknown origin. Int J Infect Dis. 2014;19:53–58. 7. Zhuang H, Alavi A. 18-Fluorodeoxyglucose positron emission tomographic imaging in the detection and monitoring of infection and inflammation. Semin Nucl Med. 2002;32:47–59. 8. Arkader A, Glotzbecker M, Hosalkar HS, et al. Primary musculoskeletal Langerhans cell histiocytosis in children: an analysis for a 3-decade period. J Pediatr Orthop. 2009;29:201–207. 9. Amini B, Kumar R, Wang WL. Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients. Skeletal Radiol. 2013;42:1301–1309. 10. Islinger RB, Kuklo TR, Owens BD, et al. Langerhans' cell histiocytosis in patients older than 21 years. Clin Orthop Relat Res. 2000:231–235. 11. Prosch H, Grois N, Prayer D, et al. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer. 2004;43:594–599. 12. Lee HJ, Ahn BC, Lee SW, et al. The usefulness of F-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Langerhans cell histiocytosis. Ann Nucl Med. 2012;26:730–737. 13. Kaste SC, Rodriguez-Galindo C, McCarville ME, et al. PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 2007;37:615–622. 14. Giovanella L, Ceriani L, Crippa S, et al. Imaging in endocrinology: Langherans cell histiocytosis of the thyroid gland detected by 18FDG-PET/CT. J Clin Endocrinol Metab. 2007;92:2866–2867. 15. Mueller RJ, Siegel A, Seltzer M, et al. Langerhans cell histiocytosis of the auditory canal detected by 18F-FDG PET/CT. Clin Nucl Med. 2012;37:908–909. 16. Caoduro C, Ungureanu CM, Rudenko B, et al. 18F-fluoride PET/CT aspect of an unusual case of Erdheim-Chester disease with histologic features of Langerhans cell histiocytosis. Clin Nucl Med. 2013;38:541–542. 17. Fotos JS, Flemming DJ, Tulchinsky M. False-positive PET/CT for lymphoma recurrence secondary to Langerhans cell histiocytosis. Clin Nucl Med. 2011;36:717–719. 18. De Souza B, Brunetti A, Fulham MJ, et al. Pituitary microadenomas: a PET study. Radiology. 1990;177:39–44. 19. Agarwal KK, Sharma P, Singla S, et al. A rare case of non-small cell lung cancer metastasizing to the pituitary gland: detection with (18)F-FDG PET-CT. Clin Nucl Med. 2014;39:e318–e319. 20. Hayasaka K, Koyama M, Yamashita T. Primary pituitary lymphoma diagnosis by FDG-PET/CT. Clin Nucl Med. 2010;35:205. 21. Jeong SY, Lee SW, Lee HJ, et al. Incidental pituitary uptake on whole-body 18F-FDG PET/CT: a multicentre study. Eur J Nucl Med Mol Imaging. 2010;37:2334–2343. 22. Zhao X, Xiao J, Xing B, et al. Comparison of (68)Ga DOTATATE to 18F-FDG uptake is useful in the differentiation of residual or recurrent pituitary adenoma from the remaining pituitary tissue after transsphenoidal adenomectomy. Clin Nucl Med. 2014;39:605–608. 23. Aide N, Benayoun M, Kerrou K, et al. Impact of [18F]-fluorodeoxyglucose ([18F]-FDG) imaging in sarcoidosis: unsuspected neurosarcoidosis discovered by [18F]-FDG PET and early metabolic response to corticosteroid therapy. Br J Radiol. 2007;80:e67–e71. 24. Hou Y, Zhu Z, Jin X, et al. Combined 18F-FDG PET/CT and 99mTc 3PRGD2 SPECT/ CT imaging in a case of pituitary metastases. Clin Nucl Med. 2013;38:550–552.
Clinical Nuclear Medicine • Volume 40, Number 6, June 2015 Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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509
Ju et al
Clinical Nuclear Medicine • Volume 40, Number 6, June 2015
FIGURE 1. A 37-year-old woman was admitted to our hospital with complaints of polydipsia and dieresis. A diagnosis of central diabetes insipidus was made after thorough examinations, which include water deprivation test. On admission, the patient's temperature was higher than normal, which ranged between 38°C and 39°C most of the time. FDG PET/CT examination was performed to find the cause of the fever.1–7 The MIP image of the PET (A) demonstrated widespread abnormal FDG activity in the lymph nodes in the bilateral neck, bilateral hilum of the lungs, mediastinum, retroperitoneal, iliac, and inguinal regions with an SUVmax of 18.6. In addition, there was diffuse elevated activity with an SUVmax of 8.0 in an enlarged spleen. Interestingly, PET images of the brain (B and C) revealed significantly elevated FDG activity with an SUVmax of 26 (black arrows) in the region of the infundibular stalk, which was confirmed by the corresponding CT (D and E) and fusion (F and G) images (white arrows).
510
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© 2014 Wolters Kluwer Health, Inc. All rights reserved. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Clinical Nuclear Medicine • Volume 40, Number 6, June 2015
Pituitary Involvement of Langerhans Cell
FIGURE 2. Both an ultrasound-guided biopsy of the cervical lymph nodes and a supraorbital keyhole approach of pituitary stalk biopsy were performed. The pathological examination (A: hematoxylin-eosin, original magnification 100; B: hematoxylin-eosin, original magnification 400) showed numerous Langerhans cells that are polygonal cells with eosinophilic cytoplasm, distributed in patches, and of medium size. The boundaries between the cells were not clear. The nuclei are oval or reniform. The further immunohistochemical staining examination showed that the tissues were vimentin (+), S-100 (+), langerin (+), CDla (+), Kp-1 (+), PGM-1 (+), and Ki67 (30%+). These findings are consistent with Langerhans cell histiocytosis. The patient received chemotherapy for Langerhans cell histiocytosis, and her symptoms of diabetes insipidus slowly improved. The most common sites of involvement of Langerhans cell histiocytosis are the bones in children.8–10 However, in our case, most lesions are located in the lymph nodes of an adult. Pituitary involvement of Langerhans cell histiocytosis is the most common cause of systemic disease, which results in central diabetes insipidus.11 FDG PET/CT findings of Langerhans cell histiocytosis have been widely described before.12–17 In addition, it is known that many different etiologies can cause elevated FDG activity in the pituitary gland.18–24 Our case indicates that Langerhans cell histiocytosis should also be considered among the differential diagnoses of abnormal pituitary FDG activity, especially when the patient has diabetes insipidus.
© 2014 Wolters Kluwer Health, Inc. All rights reserved. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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Pituitary Involvement of Langerhans Cell Histiocytosis in an Adult Unveiled by FDG PET/CT Huijun Ju, MD,* Yu Pan, MD,* Jing Lv, MD,* Xinwu Mao, MD,† and Yifan Zhang, MD, PhD* Abstract: A 39-year-old woman with complains of polydipsia and dieresis was admitted to endocrinology service for evaluation and potential treatment of diabetes insipidus. During the hospitalization, the patient was found to have unexplained fever, for which an FDG PET/CT was performed to explore the source. The images revealed multiple regions of abnormal hypermetabolism throughout the body. Notably, there was an intense activity in the region of pituitary stalk. Pathological results after biopsy showed pituitary involvement of Langerhans cell histiocytosis. Key Words: Langerhans cell histiocytosis, pituitary, FDG PET/CT (Clin Nucl Med 2015;40: 509–511)
Received for publication September 25, 2014; revision accepted October 27, 2014. From the Departments of *Nuclear Medicine, and †Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, People's Republic of China. Conflicts of interest and sources of funding: none declared. Reprints: Yifan Zhang, MD, PhD, Department of Nuclear Medicine Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No. 197, Rui Jin 2nd Rd, Shanghai 200025, People's Republic of China. E-mail: [email protected]. Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0363-9762/15/4006–0509
REFERENCES 1. Buch-Olsen KM, Andersen RV, Hess S, et al. 18F-FDG-PET/CT in fever of unknown origin: clinical value. Nucl Med Commun. 2014;35:955–960. 2. Kjaer A, Lebech AM, Eigtved A, et al. Fever of unknown origin: prospective comparison of diagnostic value of 18F-FDG PET and 111In-granulocyte scintigraphy. Eur J Nucl Med Mol Imaging. 2004;31:622–626. 3. Ma Y, Li F, Chen L. Widespread hypermetabolic lesions due to multicentric form of Castleman disease as the cause of fever of unknown origin revealed by FDG PET/CT. Clin Nucl Med. 2013;38:835–837. 4. Meller J, Sahlmann CO, Scheel AK. 18F-FDG PET and PET/CT in fever of unknown origin. J Nucl Med. 2007;48:35–45. 5. Solav SV. FDG PET/CT in evaluation of pyrexia of unknown origin. Clin Nucl Med. 2011;36:e81–e86. 6. Tokmak H, Ergonul O, Demirkol O, et al. Diagnostic contribution of (18)F-FDGPET/CT in fever of unknown origin. Int J Infect Dis. 2014;19:53–58. 7. Zhuang H, Alavi A. 18-Fluorodeoxyglucose positron emission tomographic imaging in the detection and monitoring of infection and inflammation. Semin Nucl Med. 2002;32:47–59. 8. Arkader A, Glotzbecker M, Hosalkar HS, et al. Primary musculoskeletal Langerhans cell histiocytosis in children: an analysis for a 3-decade period. J Pediatr Orthop. 2009;29:201–207. 9. Amini B, Kumar R, Wang WL. Soft tissue Langerhans cell histiocytosis with secondary bone involvement in extremities: evolution of lesions in two patients. Skeletal Radiol. 2013;42:1301–1309. 10. Islinger RB, Kuklo TR, Owens BD, et al. Langerhans' cell histiocytosis in patients older than 21 years. Clin Orthop Relat Res. 2000:231–235. 11. Prosch H, Grois N, Prayer D, et al. Central diabetes insipidus as presenting symptom of Langerhans cell histiocytosis. Pediatr Blood Cancer. 2004;43:594–599. 12. Lee HJ, Ahn BC, Lee SW, et al. The usefulness of F-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Langerhans cell histiocytosis. Ann Nucl Med. 2012;26:730–737. 13. Kaste SC, Rodriguez-Galindo C, McCarville ME, et al. PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 2007;37:615–622. 14. Giovanella L, Ceriani L, Crippa S, et al. Imaging in endocrinology: Langherans cell histiocytosis of the thyroid gland detected by 18FDG-PET/CT. J Clin Endocrinol Metab. 2007;92:2866–2867. 15. Mueller RJ, Siegel A, Seltzer M, et al. Langerhans cell histiocytosis of the auditory canal detected by 18F-FDG PET/CT. Clin Nucl Med. 2012;37:908–909. 16. Caoduro C, Ungureanu CM, Rudenko B, et al. 18F-fluoride PET/CT aspect of an unusual case of Erdheim-Chester disease with histologic features of Langerhans cell histiocytosis. Clin Nucl Med. 2013;38:541–542. 17. Fotos JS, Flemming DJ, Tulchinsky M. False-positive PET/CT for lymphoma recurrence secondary to Langerhans cell histiocytosis. Clin Nucl Med. 2011;36:717–719. 18. De Souza B, Brunetti A, Fulham MJ, et al. Pituitary microadenomas: a PET study. Radiology. 1990;177:39–44. 19. Agarwal KK, Sharma P, Singla S, et al. A rare case of non-small cell lung cancer metastasizing to the pituitary gland: detection with (18)F-FDG PET-CT. Clin Nucl Med. 2014;39:e318–e319. 20. Hayasaka K, Koyama M, Yamashita T. Primary pituitary lymphoma diagnosis by FDG-PET/CT. Clin Nucl Med. 2010;35:205. 21. Jeong SY, Lee SW, Lee HJ, et al. Incidental pituitary uptake on whole-body 18F-FDG PET/CT: a multicentre study. Eur J Nucl Med Mol Imaging. 2010;37:2334–2343. 22. Zhao X, Xiao J, Xing B, et al. Comparison of (68)Ga DOTATATE to 18F-FDG uptake is useful in the differentiation of residual or recurrent pituitary adenoma from the remaining pituitary tissue after transsphenoidal adenomectomy. Clin Nucl Med. 2014;39:605–608. 23. Aide N, Benayoun M, Kerrou K, et al. Impact of [18F]-fluorodeoxyglucose ([18F]-FDG) imaging in sarcoidosis: unsuspected neurosarcoidosis discovered by [18F]-FDG PET and early metabolic response to corticosteroid therapy. Br J Radiol. 2007;80:e67–e71. 24. Hou Y, Zhu Z, Jin X, et al. Combined 18F-FDG PET/CT and 99mTc 3PRGD2 SPECT/ CT imaging in a case of pituitary metastases. Clin Nucl Med. 2013;38:550–552.
Clinical Nuclear Medicine • Volume 40, Number 6, June 2015 Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
www.nuclearmed.com
509
Ju et al
Clinical Nuclear Medicine • Volume 40, Number 6, June 2015
FIGURE 1. A 37-year-old woman was admitted to our hospital with complaints of polydipsia and dieresis. A diagnosis of central diabetes insipidus was made after thorough examinations, which include water deprivation test. On admission, the patient's temperature was higher than normal, which ranged between 38°C and 39°C most of the time. FDG PET/CT examination was performed to find the cause of the fever.1–7 The MIP image of the PET (A) demonstrated widespread abnormal FDG activity in the lymph nodes in the bilateral neck, bilateral hilum of the lungs, mediastinum, retroperitoneal, iliac, and inguinal regions with an SUVmax of 18.6. In addition, there was diffuse elevated activity with an SUVmax of 8.0 in an enlarged spleen. Interestingly, PET images of the brain (B and C) revealed significantly elevated FDG activity with an SUVmax of 26 (black arrows) in the region of the infundibular stalk, which was confirmed by the corresponding CT (D and E) and fusion (F and G) images (white arrows).
510
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© 2014 Wolters Kluwer Health, Inc. All rights reserved. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Clinical Nuclear Medicine • Volume 40, Number 6, June 2015
Pituitary Involvement of Langerhans Cell
FIGURE 2. Both an ultrasound-guided biopsy of the cervical lymph nodes and a supraorbital keyhole approach of pituitary stalk biopsy were performed. The pathological examination (A: hematoxylin-eosin, original magnification 100; B: hematoxylin-eosin, original magnification 400) showed numerous Langerhans cells that are polygonal cells with eosinophilic cytoplasm, distributed in patches, and of medium size. The boundaries between the cells were not clear. The nuclei are oval or reniform. The further immunohistochemical staining examination showed that the tissues were vimentin (+), S-100 (+), langerin (+), CDla (+), Kp-1 (+), PGM-1 (+), and Ki67 (30%+). These findings are consistent with Langerhans cell histiocytosis. The patient received chemotherapy for Langerhans cell histiocytosis, and her symptoms of diabetes insipidus slowly improved. The most common sites of involvement of Langerhans cell histiocytosis are the bones in children.8–10 However, in our case, most lesions are located in the lymph nodes of an adult. Pituitary involvement of Langerhans cell histiocytosis is the most common cause of systemic disease, which results in central diabetes insipidus.11 FDG PET/CT findings of Langerhans cell histiocytosis have been widely described before.12–17 In addition, it is known that many different etiologies can cause elevated FDG activity in the pituitary gland.18–24 Our case indicates that Langerhans cell histiocytosis should also be considered among the differential diagnoses of abnormal pituitary FDG activity, especially when the patient has diabetes insipidus.
© 2014 Wolters Kluwer Health, Inc. All rights reserved. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
www.nuclearmed.com
511
