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Case 3

Clinical features :
Young female patient, 21 years old. Diffuse goiter with cervical adenomegaly ; high titer of antithyroid antibodies. She was treated during 2 years for auto-immune thyroiditis.
In september 2001 : FNA of cervical lymph node : papillary carcinoma metastasis.

Gross features :
Total thyroidectomy 34 grs, without obvious tumoral focus, but hard diffuse enlargment with multiple white punctiform foci.
Bilateral extensive lymphadenectomy.

Microscopy :
The entire 2 lobes are infiltrated by a diffuse proliferation, often situated in cleft-like spaces, suggesting lymphatic channels (fig A).   
   fig. A
fig. B
This tumor is made of micropapillary structures, extensive squamous metaplasia, (fig C) psammomas bodies, lymphocytic thyroiditis and prominent fibrosis (fig D).
fig. C
Some foci are in extra thyroidal situation.
25/59 lymph nodes were invaded.
fig. D

  

Diagnosis :
Diffuse sclerosing papillary thyroid carcinoma, stage pT4 - N1

Comments :
DSPC is a rare variant occuring in young people (child, adolescent, young adult, often female). This variant was frequent in Chernobyl 's children cases.
The clinical diagnosis can be delayed, confusing with thyroiditis : auto antibodies are found in 50%.
Histopathologic pattern is very typical as well in frozen sections by the association of different features in cleft -like channels. In less half of the cases, a dominant nodule is found.
The only differential diagnosis is metastatic lymphangitis carcinoma involving one or two lobes. This differential diagnosis must be evocated especially in older patient : we have seen such a case coming from bronchial carcinoma in a 36 year old male patient.
The prognosis of this variant is estimated more aggressive than classical form in term of local recurrence (cervical lymph nodes) and occurrence of pulmonary metastases, but the death rate is not higher.

References :

CHAN J.K.C., TSUI M.S., TSE C.H.
Diffuse sclerosing variant of papillary carcinoma of the thyroid : a histological and immunohistochemical study of three cases.
Histopathology 1987 ; 11 : 191-201.

CARCANGIU M.L., BIANCHI S.
Diffuse sclerosing variant of papillary thyroid carcinoma : clinicopathologic study of 15 cases.
Am J Surg Pathol 1989 ; 3 : 1041-1049.

  


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