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Fine-Needle Aspiration Biopsy of Langerhans Cell Histiocytosis of Thyroid Gland Jutarat Sangtian, Tanawan Riangwiwat, Siripich Triamchaisri, Wasana Kanoksil, Chutintorn Sriphrapradang Departments of Medicine (J.S., T.R., S.T., C.S.) and Pathology (W.K.), Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand
54-year-old postmenopausal woman presented with progressive thyroid enlargement for 1 year. Thyroid ultrasonography showed multinodular enlargement with an 8 ⫻ 6-cm isoechoic mass and a 6 ⫻ 5-cm hyperechoic mass on the right and left lobes, respectively. Thyroid function testing, including free T4 of 0.48 ng/dL (normal range, 0.7–1.48), free T3 of 3.12 pg/mL (1.71–3.71), and TSH of 5.39 mIU/L (0.35– 4.94), showed primary thyroid failure with an insufficient pituitary response, suggesting both primary and secondary hypothyroidism. Fine-needle aspiration biopsy (FNAB) was performed on the right lobe and reported as Langerhans cell histiocytosis (LCH) (Figure 1). The diagnosis of LCH is confirmed by positive immunohistochemical study for S-100 and CD1a. Further investigations revealed hypopituitarism: morning cortisol, 2.5 g/dL (5–25); FSH, 3.92 IU/L (26 –133); LH, 0.14 IU/L (10 –26); and IGF-1, 55 ng/mL (87–238). An elevated prolactin of 139.17 ng/mL (5.18 –26.53) suggested a prolactinoma, infundibular compression, or bilateral median eminence dysfunction. Central diabetes insipidus was diagnosed based on polyuria, polydipsia, depressed urine osmolarity of 113 mOsm/kg, and elevated serum osmolarity of 295 mOsm/kg. Magnetic resonance imaging pituitary showed a well-defined mass of 1.2 ⫻ 0.8 ⫻ 1 cm involving median eminence of the hypothalamus, superior aspect of the pituitary stalk, extending to the floor of the hypothalamus (Figure 2). The final diagnosis was multisystem LCH. The patient was administered chemotherapy of vinblastine 6 mg/m2 every week, prednisolone 40 mg/m2 for 4 weeks then tapered down, and levothyroxine 50 g/d. After seven cycles of chemotherapy, goiter size had decreased to 20 g by palpation, and symptoms of central diabetes insipidus had improved.
A
ISSN Print 0021-972X ISSN Online 1945-7197 Printed in U.S.A. Copyright © 2015 by the Endocrine Society Received July 7, 2014. Accepted October 8, 2014. First Published Online October 20, 2014
doi: 10.1210/jc.2014-2859
Figure 1. A, FNAB thyroid was obtained using a 25-gauge needle and smeared onto slides. Cytology showed Langerhans cell proliferation with enlarged vesicular nuclei, micronuclei, and a moderate amount of pale cytoplasm. These cells show a characteristic nuclear groove (arrow). Other cells seen on the smear included scattered eosinophils (bilobe nuclei), lymphocytes, and plasma cells. No thyroid follicular cells were seen (Papanicolaou stain). B, Ultrasonography thyroid. C and D, Computed tomography showed thyroid enlargement (arrow).
LCH mostly involved bone, skin, pituitary, liver, spleen, lungs, lymph nodes, and the central nervous system (1). The most common endocrine manifestation of LCH is central diabetes insipidus. LCH of the thyroid is extremely rare and can be presented in localized or multisystem disease (2, 3). Abbreviations: FNAB, fine-needle aspiration biopsy; LCH, Langerhans cell histiocytosis.
J Clin Endocrinol Metab, January 2015, 100(1):15–16
jcem.endojournals.org
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 11 January 2015. at 16:08 For personal use only. No other uses without permission. . All rights reserved.
15
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Sangtian et al
FNAB Thyroid Langerhans Cell Histiocytosis
J Clin Endocrinol Metab, January 2015, 100(1):15–16
Acknowledgments Address all correspondence and requests for reprints to: Chutintorn Sriphrapradang, MD, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama 6 Road, Rajthevi, Bangkok 10400, Thailand. E-mail: [email protected]. Disclosure Summary: The authors have nothing to disclose.
References 1. Haupt R, Minkov M, Astigarraga I, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175–184. 2. Makras P, Alexandraki KI, Chrousos GP, Grossman AB, Kaltsas GA. Endocrine manifestations in Langerhans cell histiocytosis. Trends Endocrinol Metab. 2007;18(6):252–257. 3. Lieberman PH, Jones CR, Steinman RM, et al. Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol. 1996;20(5):519 –552.
Figure 2. A, Skull x-ray showed small osteolytic lesion at the frontal bone (arrow). B and C, Fat suppressed T1-weighted magnetic resonance imaging pituitary with gadolinium showed a homogenous enhancing well-defined lesion of 1.2 ⫻ 0.8 ⫻ 1 cm involving median eminence of the hypothalamus, superior aspect of pituitary stalk, extending to floor of the hypothalamus (arrow).
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 11 January 2015. at 16:08 For personal use only. No other uses without permission. . All rights reserved.
i n
F E A T U R E
E n d o c r i n o l o g y
Fine-Needle Aspiration Biopsy of Langerhans Cell Histiocytosis of Thyroid Gland Jutarat Sangtian, Tanawan Riangwiwat, Siripich Triamchaisri, Wasana Kanoksil, Chutintorn Sriphrapradang Departments of Medicine (J.S., T.R., S.T., C.S.) and Pathology (W.K.), Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand
54-year-old postmenopausal woman presented with progressive thyroid enlargement for 1 year. Thyroid ultrasonography showed multinodular enlargement with an 8 ⫻ 6-cm isoechoic mass and a 6 ⫻ 5-cm hyperechoic mass on the right and left lobes, respectively. Thyroid function testing, including free T4 of 0.48 ng/dL (normal range, 0.7–1.48), free T3 of 3.12 pg/mL (1.71–3.71), and TSH of 5.39 mIU/L (0.35– 4.94), showed primary thyroid failure with an insufficient pituitary response, suggesting both primary and secondary hypothyroidism. Fine-needle aspiration biopsy (FNAB) was performed on the right lobe and reported as Langerhans cell histiocytosis (LCH) (Figure 1). The diagnosis of LCH is confirmed by positive immunohistochemical study for S-100 and CD1a. Further investigations revealed hypopituitarism: morning cortisol, 2.5 g/dL (5–25); FSH, 3.92 IU/L (26 –133); LH, 0.14 IU/L (10 –26); and IGF-1, 55 ng/mL (87–238). An elevated prolactin of 139.17 ng/mL (5.18 –26.53) suggested a prolactinoma, infundibular compression, or bilateral median eminence dysfunction. Central diabetes insipidus was diagnosed based on polyuria, polydipsia, depressed urine osmolarity of 113 mOsm/kg, and elevated serum osmolarity of 295 mOsm/kg. Magnetic resonance imaging pituitary showed a well-defined mass of 1.2 ⫻ 0.8 ⫻ 1 cm involving median eminence of the hypothalamus, superior aspect of the pituitary stalk, extending to the floor of the hypothalamus (Figure 2). The final diagnosis was multisystem LCH. The patient was administered chemotherapy of vinblastine 6 mg/m2 every week, prednisolone 40 mg/m2 for 4 weeks then tapered down, and levothyroxine 50 g/d. After seven cycles of chemotherapy, goiter size had decreased to 20 g by palpation, and symptoms of central diabetes insipidus had improved.
A
ISSN Print 0021-972X ISSN Online 1945-7197 Printed in U.S.A. Copyright © 2015 by the Endocrine Society Received July 7, 2014. Accepted October 8, 2014. First Published Online October 20, 2014
doi: 10.1210/jc.2014-2859
Figure 1. A, FNAB thyroid was obtained using a 25-gauge needle and smeared onto slides. Cytology showed Langerhans cell proliferation with enlarged vesicular nuclei, micronuclei, and a moderate amount of pale cytoplasm. These cells show a characteristic nuclear groove (arrow). Other cells seen on the smear included scattered eosinophils (bilobe nuclei), lymphocytes, and plasma cells. No thyroid follicular cells were seen (Papanicolaou stain). B, Ultrasonography thyroid. C and D, Computed tomography showed thyroid enlargement (arrow).
LCH mostly involved bone, skin, pituitary, liver, spleen, lungs, lymph nodes, and the central nervous system (1). The most common endocrine manifestation of LCH is central diabetes insipidus. LCH of the thyroid is extremely rare and can be presented in localized or multisystem disease (2, 3). Abbreviations: FNAB, fine-needle aspiration biopsy; LCH, Langerhans cell histiocytosis.
J Clin Endocrinol Metab, January 2015, 100(1):15–16
jcem.endojournals.org
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 11 January 2015. at 16:08 For personal use only. No other uses without permission. . All rights reserved.
15
16
Sangtian et al
FNAB Thyroid Langerhans Cell Histiocytosis
J Clin Endocrinol Metab, January 2015, 100(1):15–16
Acknowledgments Address all correspondence and requests for reprints to: Chutintorn Sriphrapradang, MD, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama 6 Road, Rajthevi, Bangkok 10400, Thailand. E-mail: [email protected]. Disclosure Summary: The authors have nothing to disclose.
References 1. Haupt R, Minkov M, Astigarraga I, et al. Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer. 2013;60(2):175–184. 2. Makras P, Alexandraki KI, Chrousos GP, Grossman AB, Kaltsas GA. Endocrine manifestations in Langerhans cell histiocytosis. Trends Endocrinol Metab. 2007;18(6):252–257. 3. Lieberman PH, Jones CR, Steinman RM, et al. Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol. 1996;20(5):519 –552.
Figure 2. A, Skull x-ray showed small osteolytic lesion at the frontal bone (arrow). B and C, Fat suppressed T1-weighted magnetic resonance imaging pituitary with gadolinium showed a homogenous enhancing well-defined lesion of 1.2 ⫻ 0.8 ⫻ 1 cm involving median eminence of the hypothalamus, superior aspect of pituitary stalk, extending to floor of the hypothalamus (arrow).
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 11 January 2015. at 16:08 For personal use only. No other uses without permission. . All rights reserved.
