L. Ionescu, C. Ungureanu, C. Radulescu, D. Guta, I. Trifescu, C. Vulpoi University of Medicine and...
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Transcript of L. Ionescu, C. Ungureanu, C. Radulescu, D. Guta, I. Trifescu, C. Vulpoi University of Medicine and...
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.