British Journal of Radiology (2006) 79, 703-705
© 2006 British Institute of Radiology
doi: 10.1259/bjr/80615044
A young female with galactorrhoea and sudden onset chest pain
C J Das, MD, DNB1,
R Sharma, MD1,
A Seith, MD1 and
M P Baruah, DM2
Departments of 1Radiodiagnosis and 2Endocrinology, All India Institute of Medical Sciences, New Delhi-29, India
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Introduction
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A 27-year-old married woman presented to the gynaecology out-patient department with a 3 month history of galactorrhoea and irregular menstrual cycle. She also complained of polyuria and polydipsia. No history of visual problems could be elicited. After initial evaluation, she was referred to the endocrinology department for further investigation of her hormonal status. Clinical examination was essentially within normal limits except for the galactorrhoea. Her pertinent laboratory investigations included high serum prolactin level (150 ng ml–1), low levels of gonadotropins (undetectable/ELISA), cortisol (3.8 µg ml–1) and thyroid stimulating hormone (3.8 µg ml–1), suggesting panhypopituitarism. A high resolution contrast enhanced MRI (CEMRI) of the sella was performed. MRI revealed a soft tissue mass in the suprasellar region involving hypothalamus, tuber cinerium and extending into the pituitary stalk causing its expansion. The mass was hypointense on T1 weighted images (Figure 1a
) and hyperintense on T2 weighted images (Figure 1b) showing intense homogeneous enhancement on post-gadolinium images (Figure 1c). 2 months later, the patient presented with one episode of sudden onset dyspnoea and severe non-productive cough. A posteroanterior (PA) chest radiograph revealed reticular opacities and pneumothorax on the left side (Figure 2). What is the possible diagnosis with this clinical presentation and radiological appearance?
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Figure 1. Contrast enhanced MRI sella showing soft tissue mass in suprasellar region involving hypothalamus, tuber cinerium extending into the pituitary stalk, which is hypointense on(a) T1 weighted images and hyperintense on (b) T2 weighted images, showing intense homogeneous enhancement on (c) post-gadolinium images.
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Figure 2. Chest radiograph posteroanterior view revealed reticular opacities and pneumothorax on the left side.
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Discussion
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A provisional diagnosis of tuberculoma was suggested, based on the MR appearance and in view of the high prevalence of this disease in India. The patient was put on anti-tubercular treatment, but her clinical condition did not improve even after 12 months and a repeat CEMRI (Figure 3) at this time did not show any change in the size of the suprasellar mass. In view of the history of sudden onset dyspnoea and pneumothorax seen on the chest radiograph, a high resolution CT (HRCT) scan of chest was performed which revealed multiple bizarrely-shaped cystic lesions in both lungs (Figure 4). Skeletal survey did not reveal any bony lesions. The diagnosis was reviewed and, based on the clinical presentation as well as MR and HRCT appearance, a final diagnosis of pulmonary Langerhans' cell histiocytosis (PLCH) with involvement of the hypothalamic-pituitary axis causing panhypopituitarism and diabetes insipidus was made. A thoracoscopic lung biopsy was performed and histopathology confirmed the diagnosis of PLCH. The patient was treated with hormone replacement therapy for panhypopituitarism and Carbamazapine for diabetes insipidus. PLCH was treated with a combination of chemotherapy and radiotherapy to which she responded. On clinical follow-up the patient is doing well.
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Figure 3. (a,b) Follow-up contrast enhanced MRI after anti-tubercular treatment does not show any change in the size of the suprasellar mass.
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Figure 4. High resolution CT(HRCT) scan of chest shows multiple bizarrely-shaped cystic lesions in both lungs.
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PLCH is an uncommon disorder of unknown aetiology that typically affects young adults who are smokers [1]. Previously called histiocytosis X, this is a group of diseases characterized by proliferation of histiocytes [1]. There are unifocal and multifocal forms of this disease. The unifocal disease shows solitary lytic bone lesion. This form spares the hypothalamic pituitary axis. Adult PLCH occurs most commonly as a disease isolated to the lungs. However, adult PLCH can occur as a part of the Hand-Schuller-Christian syndrome – a triad of diabetic insipidus, exophthalmos and lytic bone lesions which is commonly seen in children [1]. Taking LCH as a whole, about one-third of cases are seen in adults and 16–34% of these have pulmonary involvement [2]. In the past, PLCH was regarded as a disease with male predominance, but is now considered to be equally prevalent in both sexes [3], a change that possibly reflects altered smoking habits. Diabetes insipidus is the most common endocrinopathy reported with adult PLCH with the prevalence being about 5–9% [2]. Other endocrine abnormalities include growth hormone deficiency, hypothyroidism and abnormal glucose tolerance [4, 5].
The characteristic radiographic appearance of PLCH consists of diffuse, symmetric nodular/reticulonodular opacities predominantly in the mid and upper zones with relative sparing of the lung bases [3, 6]. For pulmonary involvement, HRCT is the investigation of choice [7, 8]. The principal findings are cysts with irregular margins and nodules, often in combination, giving a characteristic appearance. These cysts are thin walled and are usually less than 10 mm in diameter. Although most of the cysts appear round, they can also have bizarre shapes, being bilobed, clover-leaf shaped, or branching in appearance. The nodules are usually solid, centrilobular in location and measure less than 5 mm in diameter [7, 8]. The main differential diagnosis of PLCH on HRCT is lymphangioleiomymatosis (LAM). The most useful differentiating features are: the distribution of cysts, which are wide spread in PLCH, decreasing towards the lower zone and sparing the costophrenic angles, but evenly diffuse in LAM; the possible presence of nodules in PLCH and their rarity in LAM; the tendency for LAM cysts to be uniformly rounded, whereas some of the cysts in PLCH are irregular [9].
CEMRI is the ideal investigation for delineation of intracranial involvement. CT and MRI show thickening and enhancement of the infundibulum and hypothalamus. The posterior pituitary bright spot may be absent. The lesions are hyperintense on T2 weighted images [1]. Differential diagnoses include tuberculosis, sarcoidosis, hypothalamic hamartoma and chiasmatic glioma. Characteristic bone lesions and a classical clinical picture aid in the diagnosis of PLCH. Treatment of PLCH is problematic because of the unpredictable and variable outcome. Abstaining from smoking may halt the disease progression. Radiotherapy and chemotherapy may be needed for the intraocular involvement. Lung transplantation for terminal respiratory insufficiency has also been advocated [10].
Received for publication March 7, 2005.
Revision received July 12, 2005.
Accepted for publication August 16, 2005.
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References
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- Wilson AG. Pulmonary diseases of unknown origin and miscellaneous lung disorders in Peter Armstrong's Imaging of diseases of the chest, 3rd edn. Mosby, 2000. 655–60.
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Introduction
Discussion
