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Langerhans cell histiocytosis with thyroid involvement
EUROPEANJOURNALOF
RADIOLOGY European Journal of Radiology 22 (1996) 155-157
ELSEVIER
Case report
Langerhans cell histiocytosis with thyroid involvement V.F.H. Chong 1 Department of Diagnostic Radiology, Singapore General Hospital, Outram Road, Singapore 169608, Singapore Received 29 November 1995; revised 16 January 1996; accepted 30 January 1996
Keywords: Neoplasms, Langerhans cell histiocytosis; Neoplasms, hypothalamus; Hypothalamus, neoplasm
1. Introduction Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, with involvement of the thyroid gland is uncommon [1]. Up to 1991, there were only l0 such patients reported in the English literature [2]. An English literature search up to 1995 revealed a further six reports [3-8]. Respiratory embarrassment as a result of thyroid gland enlargement and tracheal compression is even more uncommon. This was documented in two previous papers [2,9]. This report documents the computed tomography (CT) findings in the head, neck I Tel.: +65 3265031; Fax: +65 3265031.
a
and chest as well as magnetic resonance imaging (MRI) features in the brain of a patient with LCH and respiratory distress as a result of thyroid gland enlargement. 2. Case report The patient, a 19-year-old female, presented at the Accident and Emergency Department with cyanosis and respiratory distress. She had stridor for 2 days prior to admission. Physical examination showed an enlarged thyroid gland. She was known to have diabetes insipidus for the past 3 years and was noted to have an enlarged thyroid gland 5 months prior to the current admission. The admission chest radiograph showed widespread
b
c
Fig. I. (a) Frontal chest radiograph taken at admission in the accident and emergency department shows widespread alveolar shadowing indicating acute pulmonary oedema. (b) Axial CT shows a diffusely enlarged thyroid gland (*) with compression of the trachea (arrowheads). Lateral displacement of the right carotid sheath is noted (arrow). (c) Axial CT of the chest shows cystic changes in the right upper lobe anteriorly (arrows). There is image degradation due to respiratory embarrassment. Note the residual alveolar pulmonary ocdema. 0720-048X/96/$15.00 © 1996 Elsevier Science Ireland Ltd. All fights reserved PII S0720-048X(96)00759-0
156
V. Chong/ European Journal of Radiology 22 (1996) 155-157
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a
b
c
Fig. 2. (a) Coronal CT shows in enhancing lesion in the floor of the third ventricle (arrow). (b) Coronal MRI shows an enhancing lesion in the hypothalamus (arrow). It is better seen compared to CT. (c) Sagittal MRI shows a large enhancing hypothalamic lesion (arrow) protruding into the basal cistern.
alveolar shadowing suggesting the presence of pulmonary oedema (Fig. l a). Her chest signs improved with ventilation therapy. CT of the neck showed diffuse enlargement of the thyroid gland (Fig. lb). A subsequent biopsy of the thyroid gland revealed evidence of LCH. CT was extended to cover the chest (Fig. lc). The image quality of the chest CT was degraded as the patient was rather uncooperative and had residual respiratory distress. Nevertheless, there was evidence of cystic changes. Axial and coronal CT of the brain showed an enhancing lesion in the region of the hypothalamus (Fig. 2a). The hypothalamic lesion was much better delineated on contrast enhanced MRI in the coronal and sagittal planes (Fig. 2h,c). 3. Discussion LCH is a group of clinical syndromes (Letterer Siwe disease, Hand Schuller Christian disease and eosinophilic granuloma) characterised by the proliferation of Langerhans cells [10]. Involvement of the thyroid gland is rare. The ultrasound characteristics of thyroid involvement was first described by Gaines et al. and was reported to resemble thyroiditis [2]. A review of the literature did not reveal reports on the CT features of thyroid involvement in LCH. The patient in this report showed diffuse thyroid enlargement. There was little contrast enhancement and the post contrast attenuation of the thyroid gland was similar to skeletal muscles. Diabetes insipidus is the most common endocrino-
logical abnormality in LCH [10]. Most patients with thyroid involvement (80%) appear to have concomitant diabetes insipidus [2]. This is higher than the 20-50% generally reported in LCH [11]. Granulomas in the hypothalamus may be seen on both CT or MRI. The hypothalamic lesion, however, is better delineated on MRI [12]. Thickening of the pituitary stalk may also be demonstrated [13]. The patient in this report showed an enhancing hypothalamic mass bulging into the basal cistern on MRI. The pituitary stalk was normal. LCH involving the lungs are mainly seen in young or middle-age adults [14]. CT demonstrates parenchymal changes much better than plain radiographs or conventional tomography [15]. Features that may be seen on CT includes nodules, cysts, pneumothorax and hazy ground-glass opacities. These parenchymal abnormalities appear more accurately defined by high resolution CT [16]. Cystic lesions were noted in the patient of this report. As the patient was still dyspnoeic, the quality of the scan was suboptimal. It was difficult to recognise other abnormalities. The ground-glass appearance noted in both lungs is most likely to be due to residual pulmonary oedema although such a feature may be seen in LCH especially on high-resolution CT. In conclusion, LCH involving the thyroid gland is unusual. Respiratory embarrassment secondary to tracheal compression is a rare complication. CT may show diffuse thyroid gland enlargement with narrowing of the trachea. There was no significant contrast enhancement and the density was similar to skeletal muscles.
V. Chong/ European Journal of Radiology 22 (1996) 155-157
References [1] Avery ME, McAfee JG, Guild HG. The course and prognosis of reticulo-endotheliosis (eosinophilic granuloma, SchullerChristian disease and Letterer-Siwe disease): a study of forty cases. Am J Med 1957; 22: 636-652. [2] Gaines P, Chan JCN, Cockram CS. Histiocytosis X involving the thyroid and hypothalamus. Postgrad Med J 1991; 67: 680-682. [3] Goldstein N, Layfield I.J. Thyromegaly secondary to simultaneous papillary carcinoma and histiocytosis X. Report of a case and review of the literature. Acta Cytol 1991; 35: 422-426. [4] Ho KH, Chong AP, Thai AC. Langerhans cell histiocytosis presenting as a gnitre: a case report. Ann Acad Med Singapore 1993; 22: 598-602. [5] Kirchgraber PR, Weaver MG, Arafah BM, Abdul Karim FW. Fine needle aspiration cytology of Langerhans cell histiocytosis involving the thyroid: a case report. Acta Cytol 1994; 38: 101-106. [6] Manrea S, Lastoria S, Klain M, Brunetti A, Boscaino A, Lupoli G, Salvatore M. Diagnostic evaluation of thyroid involvement by histiocytosis X. J Nucl Med 1994; 35: 263-265. [7] Tsang WY, Lau MF, Chan JK. Incidental Langerhans cell histiocytosis of the thyroid. Histopathology 1994; 24: 397-399. [8] Liu CS, Chin TW, Fahn HI, Wei CF. Histiocytosis X involving
[9]
[10]
[11] [12]
[13]
[14] [15]
[16]
157
the thyroid gland: a case report. Chung Hua I Hsieh Tsa Chih Taipci 1994; 54: 279-281. Lahey ME, Rallison ML, Hilding DA, Ater J. Involvement of the thyroid in histiocytosis X. Am J Pediatr Hematol Oncol 1986; 8: 257-259. Grundy R, Ellis R. Histiocytosis X: a review of the etiology, pathology, staging and therapy. Med Pediatr Oncol 1986; 14: 45-50. Berry DH, Becton DL. Natural history of histiocytosis X. Hematol Oncol Clin North Am 1987; 1: 23-34. Moore JB, Kulkarni R, Crutcher DC, Bhimani S. MRI in multifocai eosinophilic granuioma: staging disease and monitoring response to therapy. Am J Pediatr Haem Oncol 1989; 1I: 174-177. Tien RD, Newton TH, McDermott MW. Thickened pituitary stalk on MR images in patients with diabetes insipidus and Langerhans cell histiocytosis. AJNR 1991; 1!: 703-708. Marcy TW, Reynolds HY. Pulmonary histiocytosis X. Lung 1985; 163: 129-150. Moore ADA, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL, Takasugi JE, de Carvalho CRR. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989; 172: 249-254. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis: evaluation with high-resolution CT. Radiology 1989; 172: 255-258.
RADIOLOGY European Journal of Radiology 22 (1996) 155-157
ELSEVIER
Case report
Langerhans cell histiocytosis with thyroid involvement V.F.H. Chong 1 Department of Diagnostic Radiology, Singapore General Hospital, Outram Road, Singapore 169608, Singapore Received 29 November 1995; revised 16 January 1996; accepted 30 January 1996
Keywords: Neoplasms, Langerhans cell histiocytosis; Neoplasms, hypothalamus; Hypothalamus, neoplasm
1. Introduction Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, with involvement of the thyroid gland is uncommon [1]. Up to 1991, there were only l0 such patients reported in the English literature [2]. An English literature search up to 1995 revealed a further six reports [3-8]. Respiratory embarrassment as a result of thyroid gland enlargement and tracheal compression is even more uncommon. This was documented in two previous papers [2,9]. This report documents the computed tomography (CT) findings in the head, neck I Tel.: +65 3265031; Fax: +65 3265031.
a
and chest as well as magnetic resonance imaging (MRI) features in the brain of a patient with LCH and respiratory distress as a result of thyroid gland enlargement. 2. Case report The patient, a 19-year-old female, presented at the Accident and Emergency Department with cyanosis and respiratory distress. She had stridor for 2 days prior to admission. Physical examination showed an enlarged thyroid gland. She was known to have diabetes insipidus for the past 3 years and was noted to have an enlarged thyroid gland 5 months prior to the current admission. The admission chest radiograph showed widespread
b
c
Fig. I. (a) Frontal chest radiograph taken at admission in the accident and emergency department shows widespread alveolar shadowing indicating acute pulmonary oedema. (b) Axial CT shows a diffusely enlarged thyroid gland (*) with compression of the trachea (arrowheads). Lateral displacement of the right carotid sheath is noted (arrow). (c) Axial CT of the chest shows cystic changes in the right upper lobe anteriorly (arrows). There is image degradation due to respiratory embarrassment. Note the residual alveolar pulmonary ocdema. 0720-048X/96/$15.00 © 1996 Elsevier Science Ireland Ltd. All fights reserved PII S0720-048X(96)00759-0
156
V. Chong/ European Journal of Radiology 22 (1996) 155-157
! !
a
b
c
Fig. 2. (a) Coronal CT shows in enhancing lesion in the floor of the third ventricle (arrow). (b) Coronal MRI shows an enhancing lesion in the hypothalamus (arrow). It is better seen compared to CT. (c) Sagittal MRI shows a large enhancing hypothalamic lesion (arrow) protruding into the basal cistern.
alveolar shadowing suggesting the presence of pulmonary oedema (Fig. l a). Her chest signs improved with ventilation therapy. CT of the neck showed diffuse enlargement of the thyroid gland (Fig. lb). A subsequent biopsy of the thyroid gland revealed evidence of LCH. CT was extended to cover the chest (Fig. lc). The image quality of the chest CT was degraded as the patient was rather uncooperative and had residual respiratory distress. Nevertheless, there was evidence of cystic changes. Axial and coronal CT of the brain showed an enhancing lesion in the region of the hypothalamus (Fig. 2a). The hypothalamic lesion was much better delineated on contrast enhanced MRI in the coronal and sagittal planes (Fig. 2h,c). 3. Discussion LCH is a group of clinical syndromes (Letterer Siwe disease, Hand Schuller Christian disease and eosinophilic granuloma) characterised by the proliferation of Langerhans cells [10]. Involvement of the thyroid gland is rare. The ultrasound characteristics of thyroid involvement was first described by Gaines et al. and was reported to resemble thyroiditis [2]. A review of the literature did not reveal reports on the CT features of thyroid involvement in LCH. The patient in this report showed diffuse thyroid enlargement. There was little contrast enhancement and the post contrast attenuation of the thyroid gland was similar to skeletal muscles. Diabetes insipidus is the most common endocrino-
logical abnormality in LCH [10]. Most patients with thyroid involvement (80%) appear to have concomitant diabetes insipidus [2]. This is higher than the 20-50% generally reported in LCH [11]. Granulomas in the hypothalamus may be seen on both CT or MRI. The hypothalamic lesion, however, is better delineated on MRI [12]. Thickening of the pituitary stalk may also be demonstrated [13]. The patient in this report showed an enhancing hypothalamic mass bulging into the basal cistern on MRI. The pituitary stalk was normal. LCH involving the lungs are mainly seen in young or middle-age adults [14]. CT demonstrates parenchymal changes much better than plain radiographs or conventional tomography [15]. Features that may be seen on CT includes nodules, cysts, pneumothorax and hazy ground-glass opacities. These parenchymal abnormalities appear more accurately defined by high resolution CT [16]. Cystic lesions were noted in the patient of this report. As the patient was still dyspnoeic, the quality of the scan was suboptimal. It was difficult to recognise other abnormalities. The ground-glass appearance noted in both lungs is most likely to be due to residual pulmonary oedema although such a feature may be seen in LCH especially on high-resolution CT. In conclusion, LCH involving the thyroid gland is unusual. Respiratory embarrassment secondary to tracheal compression is a rare complication. CT may show diffuse thyroid gland enlargement with narrowing of the trachea. There was no significant contrast enhancement and the density was similar to skeletal muscles.
V. Chong/ European Journal of Radiology 22 (1996) 155-157
References [1] Avery ME, McAfee JG, Guild HG. The course and prognosis of reticulo-endotheliosis (eosinophilic granuloma, SchullerChristian disease and Letterer-Siwe disease): a study of forty cases. Am J Med 1957; 22: 636-652. [2] Gaines P, Chan JCN, Cockram CS. Histiocytosis X involving the thyroid and hypothalamus. Postgrad Med J 1991; 67: 680-682. [3] Goldstein N, Layfield I.J. Thyromegaly secondary to simultaneous papillary carcinoma and histiocytosis X. Report of a case and review of the literature. Acta Cytol 1991; 35: 422-426. [4] Ho KH, Chong AP, Thai AC. Langerhans cell histiocytosis presenting as a gnitre: a case report. Ann Acad Med Singapore 1993; 22: 598-602. [5] Kirchgraber PR, Weaver MG, Arafah BM, Abdul Karim FW. Fine needle aspiration cytology of Langerhans cell histiocytosis involving the thyroid: a case report. Acta Cytol 1994; 38: 101-106. [6] Manrea S, Lastoria S, Klain M, Brunetti A, Boscaino A, Lupoli G, Salvatore M. Diagnostic evaluation of thyroid involvement by histiocytosis X. J Nucl Med 1994; 35: 263-265. [7] Tsang WY, Lau MF, Chan JK. Incidental Langerhans cell histiocytosis of the thyroid. Histopathology 1994; 24: 397-399. [8] Liu CS, Chin TW, Fahn HI, Wei CF. Histiocytosis X involving
[9]
[10]
[11] [12]
[13]
[14] [15]
[16]
157
the thyroid gland: a case report. Chung Hua I Hsieh Tsa Chih Taipci 1994; 54: 279-281. Lahey ME, Rallison ML, Hilding DA, Ater J. Involvement of the thyroid in histiocytosis X. Am J Pediatr Hematol Oncol 1986; 8: 257-259. Grundy R, Ellis R. Histiocytosis X: a review of the etiology, pathology, staging and therapy. Med Pediatr Oncol 1986; 14: 45-50. Berry DH, Becton DL. Natural history of histiocytosis X. Hematol Oncol Clin North Am 1987; 1: 23-34. Moore JB, Kulkarni R, Crutcher DC, Bhimani S. MRI in multifocai eosinophilic granuioma: staging disease and monitoring response to therapy. Am J Pediatr Haem Oncol 1989; 1I: 174-177. Tien RD, Newton TH, McDermott MW. Thickened pituitary stalk on MR images in patients with diabetes insipidus and Langerhans cell histiocytosis. AJNR 1991; 1!: 703-708. Marcy TW, Reynolds HY. Pulmonary histiocytosis X. Lung 1985; 163: 129-150. Moore ADA, Godwin JD, Muller NL, Naidich DP, Hammar SP, Buschman DL, Takasugi JE, de Carvalho CRR. Pulmonary histiocytosis X: comparison of radiographic and CT findings. Radiology 1989; 172: 249-254. Brauner MW, Grenier P, Mouelhi MM, Mompoint D, Lenoir S. Pulmonary histiocytosis: evaluation with high-resolution CT. Radiology 1989; 172: 255-258.