5. Disseminated malignancy
Metastatic Papillary Thyroid Carcinoma
Differentiated thyroid cancer (DTC) almost exclusively presents as
papillary thyroid cancer (PTC) in children and adolescents. It is a rare
disease and it accounts for approximately 1.4% of all pediatric
malignancies1,2.
Exposure to external or internal radiation are well documented risk
factors for the development of thyroid carcinoma. Prolonged elevation of
thyroid-stimulating hormone (TSH), the presence of thyroid-stimulatory
immunoglobulins in Grave’s disease and associations with Hashimoto’s
disease have also been implicated as potential risk factors in adults.
However, direct causative relationships between these diseases and PTC
remain poorly documented, especially in the pediatric population1,2.
Children, as opposed to adults, often present with lymph node or
disseminated lung metastases (known as miliary metastases). PTC should
be suspected in children with a growing thyroidal nodule or a persistent
lymph node in the neck. Diagnostic procedures include thyroid
ultrasound, fine-needle biopsy and diagnostic hemithyroidectomy in some
cases3. Classic histopathologic findings include a typical
papillary pattern, ground glass nuclei, nuclear groove and colloid
deposition (Fig 3)4.
The absence of granulomas in tissue excluded the diagnoses of both
infectious and/or noninfectious granulomatous lung diseases such as
tuberculosis, fungal infections or sarcoidosis. Special stains and
cultures of tissue biopsy also failed to identify any pathogens as the
cause of the pulmonary nodules. The diagnosis of hypersensitivity
pneumonitis was also excluded based on the lack of features of
interstitial lymphocytic infiltrates and fibrosis, edema, noncaseating
granulomas, foamy macrophages and bronchiolitis obliterans.
Despite the relatively high rate of nodal and distant metastases,
prognosis is very favorable with long term survival rates of over 95% in
children and adolescents 3.
Since pediatric PTC is a comparatively rare disease, most treatment
options for this patient population are derived from experiences in the
adult PTC population3. Treatment options for PTC depend on the stage of the disease and degree of extrathyroid extension or lymph node metastases3.
For advanced disease with lung metastases, total thyroidectomy, node
dissection, radioiodine therapy, and TSH suppression therapy are
recommended to minimize disease recurrence4. The use of
extended surgery does increase the potential risk for surgical
complications such as recurrent laryngeal nerve (RLN) injury or
permanent hypoparathyroidism. A more conservative approach with
lobectomy for localized tumor may be a suitable option for patients with
early-stage disease3.
This patient received complete thyroidectomy followed by radioactive
iodine therapy and thyroid hormone replacement. After radioiodine
therapy, she developed respiratory insufficiency due to radiation
pneumonitis for which oral steroid therapy and supplemental oxygen were
initiated. The patient currently remains on oxygen therapy at home via
nasal cannula/face mask and has moderate exercise limitation. Chest
imaging studies still reveal signs of metastatic lung disease. Repeat
radioactive iodine therapy with the goal of achieving remission is
currently scheduled.
Reference
-
Vaisman F et al. Thyroid carcinoma in children and adolescents-systematic review of the literature. J Thyroid Res. 2011;845362.
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Grigsby PW et al. Childhood and adolescent thyroid carcinoma. Cancer. 2002 Aug 15;95(4):724-9.
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Verburg FA et al. Pediatric papillary thyroid cancer: current management challenges. Onco Targets Ther. 2016 Dec 28;10:165-175.
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Wada N et al. Pediatric differentiated thyroid carcinoma in stage I:
risk factor analysis for disease free survival. BMC Cancer. Sep 1;9:306.