Thymoma: Dr. Ravi Gadani MS, Fmas

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Thymoma

Dr. Ravi Gadani


MS, FMAS
Anatomy
• The thymus is a specialized organ of the immune
system.
• It is located in the ant. mediastinum.
• Production of T- Lymphocytes.
• A pyramid shaped organ, pinkish grey in colour
with a soft and lobulated surface.
• 5 cm in length, 4 cm in breadth and 6mm in
thickness.
Anatomy
• Weighs 20-35 gm during puberty and regresses to
6 gm in adulthood .
• 2 lobes , each lobe is composed of multiple
lobules and surrounded in a capsule .
• Each lobule consists of multiple follicle.
• Each follicle is divided into a capsule and medulla
Anatomy
• Cortex : contains thymocytes → T- lymphocytes.
• Medulla : contains corpuscles (area of
maturation).
• Supplied by Internal mammary, superior and
inferior thyroid arteries.
• The veins end in the left brachiocephalic vein
(innominate vein), and in the thyroid veins.
Mediastinal Masses
• Thymoma
• Lymphoma
• Germ cell tumor
• Thyroid and Parathyroid tumors
Thymoma
• A neoplasm of the Thymic epithelial cells.
• Results from dysregualtion of the proliferation
and maturation of T- lymphocytes.
• This process results in either Autoimmunity or
Immune deficiency .
Thymoma
• As a result, thymomas are associated with
autoimmune diseases in 70% of the patients
during diagnosis.
• Thymomas are usually encapsulated and spread
by local extension.
Epidemiology
• Primary tumors of the mediastinum represent 3%
of all chest tumors.
• Primary anterior mediastinal masses account for
50%.
• 45% are thymomas.
• F:M → 1 : 1
• Thymomas in the pediatric age group tend to run
an aggressive course.
Clinical Presentation

30% local 30% abnormal


symptoms. chest radiographs.

30% Myasthenia
Gravis
(paraneoplastic
syndrome).
Clinical Presentation
Local symptoms :
• Dyspahgea Thymomas tend to be
• Cough highly vascular →
• SVC obstruction bleeding and necrosis
• Dyspnea

Paraneoplastic :
• MG
• Hypogammaglobulenemia
• Good syndrome
• Oppurtunistic infections
Work up
• Blood :
• CBC : Anaemia, thrombocytopenia, agranulocytosis
• Quantitative Ig studies → Panhypogammaobulinenmia.
• CD4 T-cell count
Work up
• Radiology :
• Chest x ray
• CT or MRI
• Nuclear imaging (octreotide scan)
Work up
• Tissue sampling :
• Core biopsy
• FNAC
• Limited sternotomy
• Mediastinoscopy
Histologic Findings
• Mixed epithelial and lymphoid cells

• 4 categories :
• Spindle cell predominant
• Lymphocyte predominant
• Mixed
• Epithelial predominant
WHO classification
• WHO classification :
• A : Spindle or Oval cells
• B : dendritic or epitheloid
• AB : mixed
• C : resembles other organs
• Types A, AB → benign .
• Type B, C → malignant .
Staging- Masaoka Staging
Stage Description
I Macrospcopically encapsulate, no
capsular invasion
II Macroscopic invasion to surrounding
tissue or microscopic capsular invasion
III Macroscopic invasion into neighboring
organs
IVa Pleural or pericardial dissemination
IVb Lymphogenous or hematogenous
metastases
Management
• Surgical and medical

• Thymectomy is curative in the early stages

• Thymectomy releives obstructive symptoms and


improves paraneoplastic symptoms
Management
• Hypogammaglobulinemia shows no improvement
after thymectomy and requires monthly Ig
infusions.

• MG patients show a 25 % improvement in


muscular weakness after thymectomy.
Radiotherapy & Chemotherapy
• Radiotherapy : unresectable tumors & post
sugical resection.

• Primary radiotherapy for stages III & IVa


improved the 5y survival 40-50%.

• Chemotherapy : for stages Iva & IVb, using


Cisplatin , Vincristine & Doxyrubicin.
Prognosis
• Adverse predictive factors :
• Invasive tumor
• Tracheal compression
• Young age
• Tumor more than 8 cm
5 & 10 year survival
• Type A - 100% and 95%, respectively
• Type AB - 93% and 90%, respectively
• Type B1 - 89% and 85%, respectively
• Type B2 - 82% and 71%, respectively
• Type B3 - 71% and 40%, respectively
• Type C - 23% (5-year survival rate)
Thymic Hyperplasia
• Increase in the size of the gland with normal
microscopic arrangement.

• Rare entity.

• Presentation is similar to a thymoma.


Thymic Hyperplasia
• 3 subtypes :
• Massive thymic hyperplasia.
• Common in infancy presents with compressive
symptoms.
• Thymic hyperplasia assocaited with endocrine
abnormalities.
Thymic Hyperplasia
• Rebound thymic hyperplasia :
• The thymus gland regresses in size during times of
severe stress then enlargers beyond normal.
• Seen following, severe burns, pneumonia, tuberculosis
and malignancies.
Thymic Hyperplasia
• Management :
• Close monitoring for 2 years.
• If thymic hyperplasia doesn't regress by 2 years,
biopsies and resection are warranted.

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