THE DOUBLE EDGE SWORD STEROID FACILITATED DIAGNOSIS OF PRIMARY THYROID LYMPHOMA
Mudassir Ali (1), Phillip Mounter (2), Vivek Shanker (3), Shafie Kamaruddin (1)
1 Endocrinology, County Durham & Darlington Foundation Trust, DL3 6HX
2 Haematology, County Durham & Darlington Foundation Trust, DL3 6HX
3 ENT Surgery, County Durham & Darlington Foundation Trust, DL3 6HX
Introduction
Primary thyroid lymphoma (PTL) is a rare cause of malignancy, accounting for < 5% of thyroid malignancies and < 2% of extra-nodal lymphomas. Women are more commonly affected than men and typically present in the sixth or seventh decade of life. Most thyroid lymphomas are non-Hodgkin’s lymphomas of B-cell origin. Patients with Hashimoto’s thyroiditis are at greater risk for developing PTL. Early diagnosis is important as treatment and prognosis of PTL depend upon the histology and stage of the tumour at diagnosis. (1)(2)
Unlike other thyroid cancers this is not associated with radiation exposure. (1)
We report a rare case of primary thyroid lymphoma in a patient presenting with a rapidly enlarging thyroid goitre highly suspicious of anaplastic thyroid carcinoma posing as a diagnostic histological challenge due to underlying Hashimoto’s thyroiditis which was unmasked after a short course of oral steroids.
Case description
A 51 years old female was referred by GP with increasing painless swelling over the right side of neck over a period of 3 weeks (Nov 2018). She did not have any difficulty in swallowing or breathing. There was no history of any weight loss or night sweats. Her TSH was 9.57 mIU/L with a normal free T3, T4 and positive thyroid peroxidase antibody suggestive of subclinical hypothyroidism secondary to Hashimoto’s thyroiditis. The scan showed a large mass on the right side of the thyroid gland measuring 4.8 x 4.6 x 3.7 cm, encasing the common carotid artery with complete compression and thrombosis of the internal jugular vein. There were multiple lymph nodes on the right side of neck.
Initial imaging was highly suspicious of anaplastic thyroid cancer which carries a poor prognosis. She was referred to our thyroid surgeon and had a core biopsy which unexpectedly only showed inflammatory/reactive picture with no clear signs of malignancy. She was given oral steroids for 4 days, which decreased the size of the neck swelling followed by a core biopsy (Dec 2018), which was inconclusive. She later had another biopsy in Jan 2019 and the resultant histology showed clearance of the inflammatory picture whilst unravelling histological features of a high grade lymphoma. A diagnosis of B cell lymphoma was confirmed by the Haematologist. From a haematological perspective, giving steroids prior to diagnostic biopsy for suspected lymphoma is strongly discouraged as steroids can partially treat high-grade lymphomas, induce necrosis and render the biopsy uninterpretable.
She later had PET CT which showed bulky thyroid and bilateral cervical and right supra-clavicular lymphadenopathy. Case was discussed in the MDT and outcome was to start her on R-CHOP regimen. She had recently completed x 6 cycles of chemotherapy and a repeat PET-CT scan 3 weeks post chemotherapy (17/6/19), which reported thyroid to be of normal size but with residual FDG uptake. She then had consolidation radiotherapy based on MDT
decision, which was completed on 16th August 2019. She had a repeat PET CT 12 weeks post radiotherapy (13/11/19), which reported as Lymphoma in complete metabolic remission, diffuse symmetrical bilateral FDG uptake, felt to be more suggestive of thyroiditis. Clinically she remains asymptomatic, tolerated the chemotherapy and radiotherapy well and is being followed up in Haematology clinic every 3 months. There are no cases reported so far, where steroid helped in unmasking the actual diagnosis. A case reported in Journal of Thyroid disorders and Therapy (3) where a symptomatic patient received a course of steroid after open surgical biopsy, and had dramatic resolution with an eventual diagnosis of PTL.
Conclusion
This case highlights the unexpected paradoxical role of steroids in unmasking the histopathological diagnostic dilemma of PTL which carries a more favourable prognosis compared to anaplastic thyroid carcinoma whilst shrinking the mass. This earlier diagnosis will have an impact on overall prognosis of the disease. The British Thyroid Association guidelines for thyroid cancer highlights the need for high index of suspicion for PTL where a core or open biopsy is required to make a diagnosis at an early stage (2). However, this case highlights without the use of steroids it could have been difficult to interpret the histological findings to make a correct diagnosis.
Key points:
– In a rapidly enlarging neck mass, always think about PTL versus anaplastic cancer
– Use of steroid is controversial, but it could help in making diagnosis of PTL earlier, which ultimately changes management plan.
– Research and evidence is required regarding use of steroids in challenging cases of thyroid cancer, where diagnosis is in dilemma.
References:
1. Stein SA, Wartofsky L. Primary Thyroid Lymphoma: A Clinical Review. J Clin Endocrinol Metab [Internet]. 2013 Aug;98(8):3131–8. Available from:https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.2013-1428
2. Perros P, Boelaert K, Colley S, Evans C, Evans RM, Gerrard BA G, et al. Guidelines for the management of thyroid cancer. Clin Endocrinol (Oxf) [Internet]. 2014 Jul;81:1–122. Available from: http://doi.wiley.com/10.1111/cen.12515
3. Hafez MT, Nason RW. Proper Management of Rapidly Growing Large B-Cell Primary Thyroid Lymphoma, Case Report and Review of Literature. J Thyroid Disord Ther 2017;06. doi:10.4172/2167-7948.1000210.
]]>Introduction
Gitelman syndrome (GS) is a rare autosomal recessive renal disorder resulting from a mutation in the gene SLC12A3, which encodes for the thiazide‐sensitive sodium chloride co-transporter in the distal convoluted tubule. It is characterized by hypokalaemia, hypomagnesaemia, hypocalciuria, metabolic alkalosis, and normal or low blood pressure. Many symptoms of this rare renal disorder are related to these electrolyte abnormalities, and adversely affect the patient’s quality of life. We report a case of GS in pregnancy with episodes of symptomatic hypokalaemia requiring multiple hospital admissions for electrolyte replacement. (1)(2)
Case report:
A 27 years old lady who was known to have hypokalaemia since she was 17 years of age, presented to hospital in December, 2017 with symptomatic hypokalaemia complaining of tingling and numbness around her mouth and weakness of both lower limbs. This was her first admission in hospital related to hypokalaemia and the potassium was 2.2 mmol/L and magnesium was 0.6 mmol/L, she was found to be pregnant at this admission. Prior to this admission she was just on oral potassium citrate syrup 5 ml BD.
She received intravenous potassium and magnesium infusion and was discharged on oral supplements. Later in January 2018 she had miscarriage at 15 weeks. In December, 2018 she had another miscarriage at 11 weeks.
Between February to July 2018 she had monthly at-least once hospital admission for iv potassium and magnesium infusion. After July 2018 she required weekly intravenous potassium infusion about 80-160 mmol of KCL per admission and continued to have hospital admission once or twice a week for electrolyte replacement. Following informed consent, DNA analysis revealed two heterozygous pathogenic SLC12A3 variants c.334G>T, p.(Glu112Ter); c.2883+1G>T, confirming a molecular genetic diagnosis of GS.
She later became pregnant, making the management more challenging. Currently she is in her third trimester and this is her first pregnancy which came so far and hopefully she would have successful delivery of baby. Her current medications include oral amiloride 5 mg BD, potassium chloride 600 mg MR 5 tablets QDS, magnesium glycerophosphate 4 mmol BD and sodium tablets slow release 1200 mg TDS. She is under regular care of endocrinologist and nephrologist with special interest in renal genetics.
Discussion:
After miscarriage, the symptoms of GS often improve and patients have stable levels of potassium. Our case presents an unusual example where patient had resistant severe symptomatic hypokalaemia which persisted beyond her miscarriage, even on maximum tolerated therapy. Also this case highlights with multidisciplinary approach and close monitoring of potassium and other electrolytes, with adjustment of the individualised oral electrolyte replacement therapy such patients could have successful pregnancy outcomes.
A randomized controlled study (3) was performed in patients with GS providing evidence of efficacy of treatment with three drugs (Indomethacin, amiloride and eplerenone). Although these drugs have different mechanisms of action, they all increase plasma potassium concentration on the short term basis. Additional studies are needed to address the long-term efficacy and tolerability of these treatment options
In general, GS is a chronic condition that is usually manageable. However, as in our case severity of GS may seriously hamper daily activities and effect quality of life. The main aim is to correct electrolyte abnormalities, improve quality of life and prevent life threatening complications like ventricular arrhythmias and cardiac arrest.
Conclusion:
References:
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Gan EH, Robson W, Murphy P, Pickard R, Pearce S, Oldershaw R.
Endocr Connect. 2018 May;7(5):617-629. doi: 10.1530/EC-18-0067. Epub 2018 Apr 5.
Published November 2017
]]>Diabetologia
Published 30 November 2017
https://doi.org/10.1007/s00125-017-4503-0
]]>The Lancet. DOI: 10.1016/S0140-6736(17)33102-1
The DiRECT study, led by Newcastle University has found that almost half of the patients with Type 2 diabetes supported by their GPs on a weight loss programme were able to reverse their diabetes in a year.
This study was funded by Diabetes UK and has also received attention in the mainstream media, highlighting the quality research that takes place in our region.
]]>International Journal of Clinical Practice. 70: 843-852. 2016
DOI:10.1111/ijcp.12876
]]>J Med Genet 54 (1), 19-25. 2016
DOI: 10.1136/jmedgenet-2016-104064
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DOI: 10.1186/s12875-016-0406-2
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