L. Ionescu, C. Ungureanu, C. Radulescu, D. Guta, I. Trifescu, C. Vulpoi
University of Medicine and PharmacyIasi-2009
Thymo-Thyroid AssociationsClinical and Pathological Aspects
Association of thyroid and thymic lesions
• Hyperthyroidism - Myasthenia gravis, is sporadically reported in the literature
• Both conditions can aggravate each other• Appropriate management is made by a
multidisciplinary team judging on each individual case.
• ? What condition must be treated surgically first or if both conditions require surgery at the same time is still a matter of debate
Thymic pathology
• Thymic lymphoid hyperplasia Clinical picture – MG
• ThymomaClinical picture: - MG till 50%
- SVC syndrome
Myasthenia gravis and hyperthyroidism
• Present mainly in autoimmune thyropathy such as Grave’s disease.
• Usually myasthenia gravis is secondary to hyperthyroidism due to thymus hyperplasia
• An adequate anti-thyroid drug treatment or surgery result in remission of thymic hyperplasia.
Myasthenia gravis and hyperthyroidism
• Murakami M. et al. demonstrated the presence of thymic hyperplasia in Grave’s disease patients.
• Calculate on CT scan images the size and density of the thymus on untreated and treated Grave’s disease patients.
• The conclusion was thymic hyperplasia regresses in patients treated either with anti-thyroid drugs or by total thyroidectomy.
Myasthenia gravis and hyperthyroidism
• Yamanaka et al.- a case of Grave's disease associated with a mediastinal mass in which CT scan and MRI were suggestive for a thymoma.
• The patient underwent total thyroidectomy and thymectomy at the same time.
• Pathology report showed a thymic hyperplasia.
Myasthenia gravis and hyperthyroidism
• Nakamura T. et al. demonstrated by mediastinal biopsy, the presence of thyrotropine receptors in the hyperplasic thymus of a young patient with hyperthyroidism.
• The presence of these receptors raises the hypothesis that the thymus is also a target organ for the autoimmune aggression in Grave's disease
Thymic lesions - endocrinopathiesThe 3rd.Surgical Unit 1980-200985 thymopathies - 9 cases associated lesions• MG- Grave’s disease-3 cases• MG- Hashimoto’s disease-2 cases• Thymic HP-Hashimoto’s- hl. anaemia-1 case• Thymic carcinoma- Cushing sdr.- 1 case• MG (Thymoma) - Toxic MN goiter- 1 case• MG (Thymoma) - post rTh. Myxedema- 1case
Grave’s disease and Myasthenia gravis-case 1
JM, 33-year-old woman, The 3 rd Surgical Unit – 200410-year history of neglected Grave’s disease, anaphylactic shock to anti-thyroid drugs
2 weeks history of progressive myasthenia gravis
Thyroid gland volume - 28.9 ml, TSH-0.2mU/l, Ft4-2.6nmol/dlCT scan- diffuse compressive goiter
Myasthenia gravis
EMG-D-30%, positive antiChE test, CT scan- ? Thymic Lymphoid Hyperplasia
Treatment: neostigmine, steroids
Therapeutic decision
• Considering MG secondary to hyperthyroidism• Total thyroidectomy after 10 days Lugol
preparation• Medical treatment of MG and reassessment
after 6 months• Thymic hyperplasia might regress after
adequate treatment of Grave’s disease
Postoperative outcome
• Total thyroidectomy - august 2004• Pathology report - bilateral micropapillary
carcinoma on Basedow’s disease• Acute respiratory failure - prolonged
mechanical ventilation• Intensive care of myasthenic severe status:
anticholinesterase, steroids, plasmapheresis• Cardio-respiratory arrest on 28th post-op. day
Myasthenia gravis and Grave’s disease-case 2
• Avadanei M.Ileana, 42-year-old woman, • Grave’s disease- operated – oct. 2007 - total
thyroidectomy• Associated MG Osserman IIB - EMG- D-20%, CT
torace – thymic hyperplasia• Thymic scintigraphy – heterogenous captation • Neostigmine 3tb./day - good response
CT scan view of thymic hyperplasia
Thymic scintigraphyHeterogeneous captation
Myasthenia gravis and Grave’s diseaseCase 3
MM, 54-year-old woman9-year-history Grave’s disease 2005 - thyroid profile TSH-0,1 ui/ml, fT4 - 1,2ng/mlCT scan - diffuse goiter
MG and Grave’s disease
2005 MG EMG-D-18%, CT- heterogenous normal sized thymic region
Total thyroidectomy- 2005Thymectomy - 2006 Pathology report-thymolipoma
Postoperative outcome- myasthenic symptoms controlled with small doses of neostigmine
Myasthenia gravis and Hashimoto’s disease
2 cases
Myasthenia gravis+Hashimoto’s, case 1UD, 54-year-old woman
• 4 years history of progressive MG- dg.2004• EMG-D-20% , repeat EMG-D-25%• CT( 2005)- ant-sup. mediastinum with a fibrous - fatty tissues • Tretment-mestinon 60mg.de 3/zi, PDN-10mg every 2 days, some improvement• 2005- Hashimoto’s thyroiditis - ab. antiTPO-556UI/ml, compensated with
75ug/day Euthyrox: TSH-2uUI/ml., Ft4-1,2ng/dl• Myasthenia gravis got worse with increasing doses of AChE and CS.• CT 2008 – heterogenous thymic space.• Thymic scintigraphy - july 2008- discrete hyperfixation of 99mTc Tetrofosmin,
heterogenous, with vertical trajectory in the left paramedian anterior mediastinum
Outcome
• Op. july 2008- extended thymectomy through longitudinal sternotomy
• Pathology report- atrophic thymus with areas of folicular hyperplasia .
• Post-op.course- aggravating with ARF – prolonged mechanical ventilation.
• Intensive care treatment: ACE, CS, PPH without result.
Post operative complications
• Tracheostomy at 5 weeks postop. • Ventilator dependent infection,• Multiple eschars, • Axillary vein thrombosis.• Eso-tracheal fistula• Perforated corneal ulcer LE• MSOF - death - septembre 2008.
Surgical specimenPreop.thymic scintigraphy
Myasthenia gravis+ Hashimoto’s thyroiditisCase 2
• ML, 28-year-old woman• 6 months history of MG-Osserman IIB, and Hashimoto’s
thyroiditis.• MG-EMG-D-62%, + anticholinesterase test, CT- nodular
thymus (14/11 mm, 14/18 mm)• Hashimoto’s - AAT-TPO-76,7 (N<50)• Thymectomy - april 2008• Pathology- TLH, complete remision
•
CT scan aspect
Nodular thymus- Thymic Lymphoid Hyperplasia Surgical specimen
Thymic hyperplasia and Hashimoto’s disease and
haemolitic anemia1 case
Thymic hyperplasia+Hashimoto’s thyroiditis+autoimmune hemolytic anemia
GE, 19-year- old man, Hashimoto’s thyroiditis, hemolytic anaemia, (Hb-2,6g/dl), CT- thymoma, op. dec 2005,
pathology report - thymic lymphoid hyperplasia
GE-Hashimoto’s thyroiditisPost operative course- hl.anaemia
remitted
Thymoma with ACTH secretion
Cushing syndrome
Thymoma+ Cushing sdr.
• G. M. C., 32-year-old woman , • Diagnosed- Cushing sdr.- july 2008 (dr. C. Ungureanu) • ACTH, plasmatic and urinary cortizol – high levels (ACTH-
292pg/ml. basal plasmatic cortizol -582ng/ml and 590ng/ml at 23.00 PM, free urinary cortizol -532 mg/24 h.)
• DZ tip II• hipoKemie, metabolic alcalosis
• Abdominal CT scan, pituitary gland MRI, thyroid USS – WNL• Calcitonin, normal, CXR-normal
GM, 32-year-old woman, Cushing sdr. , ACTH -292pg/ml.(n<46). CT- anterior mediastinal mass, pericardial adhesion,
Op. sept. 2008-thymectomy+pericardectomy+mediastinal pleurectomy. Histology: well-differentiated thymic neuroendocrine carcinoma,
transcapsular invasion, pT2NxMx, Immunhistochemistry: NSE, chromogranin, synaptophizin- intense
positive, MNF116-moderate positive, Ki 67-10%, post.op. ACTH-37pg/ml. Chushing clinical aspect remitted
Myasthenia gravis and toxic multinodular goitre
1 case
AM, 46-year-old woman, 2007 multinodular goitre and myasthenia gravis
Thyroid profile (TSH-0.1 µUI/ml, fT4-1.2ng/dl), Thyroid total volume of 65.9 ml. (Prof. dr. C. Vulpoi)
Compressive goiter Retrosternal goiter
Total thyroidectomy for MNG-2007,Myasthenia gravis aggravated
Normal Chest Normal thymus
Thymic scintigraphyHypercaptation of 99mTc-tf. consistent with a thymoma
Repeat CT scanAntero- inferior mediastinal mass
Thymectomy, 6 months following TT, june 2008
Paramedian low retrosternal mass Well-encapsulated mass
Discussions
• In this case the thyroid lesion was more evident, and thus first treated while MG was erroneously considered secondary to hyperthyroidism and consequently likely to remit following total thyroidectomy.
• On thymic scintigraphy, the hyperfixation in lower anterior mediastinum raised the suspicion of thymoma,
• Pathology report of the surgical specimen (mixt thymoma - Muller-Hermelink classification or AB type - WHO classification, with capsular microscopic invasion, Masaoka II stage).
Myasthenia gravis, thymomaInflammatory pericarditis
• C T, 64-year-old woman• 8 year-history of MG, CT- evident tumour• op. 2002-thymectomy+pleurectomy• Pathology report- Invasive thymoma-Masaoka III• Post-operative radiotherapy 44 Gy,• Chemotherapy 1 year- CPh+PDN• 2003- post-radiotherapy myxedema
CT, 60 years old, thymoma+MG, Oss.IV, op. 2002, Lymphocitic thymoma (type I malignant thymoma)-Masaoka
II ( well encapsulated but microscopic capsular invasion), adhesions to left M. pleura which was resected
Radiotherapy 44 Gy, chemotherapy, 1 year CP+PDNPericarditis at 1 year postRxT
Remission of MG 5 years, 2008- AChE
POSTOPERATIVE THYMIC SCINTIGRAPHYLACK OF RADIOTRACER FIXATION IN THE ANTERIOR MEDIASTINUM
CT aspects-2009The absence of the tumour recurrence, pericarditis
2009Inflammatory pericarditis
Conclusions
• Hyperthyroidism may be associated with:– thymic hyperplasia, in which no surgical action should be
taken regarding the thymus,– thymoma, in which surgical treatment is essential.
• The thymic 99mTc tetrofosmin scintigraphy can be efficient in diagnosing the thymic lesions when conventional imaging investigations fail to confirm a clinical suspicion.
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