Thymus within Endocrinopathies

Report
Thymo-Thyroid Associations
Clinical and Pathological Aspects
L. Ionescu, C. Ungureanu, C. Radulescu,
D. Guta, I. Trifescu, C. Vulpoi
University of Medicine and Pharmacy
Iasi-2009
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
• Thymoma
Clinical 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 - endocrinopathies
The 3rd.Surgical Unit 1980-2009
85 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 graviscase 1
JM, 33-year-old woman,
The 3 rd Surgical Unit – 2004
10-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/dl
CT 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 scintigraphy
Heterogeneous captation
Myasthenia gravis and
Grave’s disease
Case 3
MM, 54-year-old
woman
9-year-history Grave’s
disease
2005 - thyroid profile
TSH-0,1 ui/ml,
fT4 - 1,2ng/ml
CT scan - diffuse goiter
MG and Grave’s disease
2005 MG
EMG-D-18%,
CT- heterogenous normal
sized thymic region
Total thyroidectomy- 2005
Thymectomy - 2006
Pathology reportthymolipoma
Postoperative outcomemyasthenic symptoms
controlled with small doses
of neostigmine
Myasthenia gravis and
Hashimoto’s disease
2 cases
Myasthenia gravis+Hashimoto’s, case 1
UD, 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 specimen
Preop.thymic scintigraphy
Myasthenia gravis+ Hashimoto’s thyroiditis
Case 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 anemia
1 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 thyroiditis
Post 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 (ACTH292pg/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 scintigraphy
Hypercaptation of 99mTc-tf. consistent with a thymoma
Repeat CT scan
Antero- 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, thymoma
Inflammatory 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+PDN
Pericarditis at 1 year postRxT
Remission of MG 5 years, 2008- AChE
POSTOPERATIVE THYMIC SCINTIGRAPHY
LACK OF RADIOTRACER FIXATION IN THE ANTERIOR MEDIASTINUM
CT aspects-2009
The absence of the tumour recurrence, pericarditis
2009
Inflammatory 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.

similar documents