Diagnosis and Treatment > Signs and Symptoms
Search for other papers by Sharmin Jahan in
Google Scholar
PubMed
Search for other papers by M A Hasanat in
Google Scholar
PubMed
Search for other papers by Tahseen Mahmood in
Google Scholar
PubMed
Search for other papers by Shahed Morshed in
Google Scholar
PubMed
Search for other papers by Raziul Haq in
Google Scholar
PubMed
Search for other papers by Md Fariduddin in
Google Scholar
PubMed
Summary
Silent corticotroph adenoma (SCA) is an unusual type of nonfunctioning pituitary adenoma (NFA) that is silent both clinically and biochemically and can only be recognized by positive immunostaining for ACTH. Under rare circumstances, it can transform into hormonally active disease presenting with severe Cushing syndrome. It might often produce diagnostic dilemma with difficult management issue if not thoroughly investigated and subtyped accordingly following surgery. Here, we present a 21-year-old male who initially underwent pituitary adenomectomy for presumed NFA with compressive symptoms. However, he developed recurrent and invasive macroadenoma with severe clinical as well as biochemical hypercortisolism during post-surgical follow-up. Repeat pituitary surgery was carried out urgently as there was significant optic chiasmal compression. Immunohistochemical analysis of the tumor tissue obtained on repeat surgery proved it to be an aggressive corticotroph adenoma. Though not cured, he showed marked clinical and biochemical improvement in the immediate postoperative period. Anticipating recurrence from the residual tumor, we referred him for cyber knife radio surgery.
Learning points:
-
Pituitary NFA commonly present with compressive symptoms such as headache and blurred vision.
-
Post-surgical development of Cushing syndrome in such a case could be either drug induced or endogenous.
-
In the presence of recurrent pituitary tumor, ACTH-dependent Cushing syndrome indicates CD.
-
Rarely a SCA presenting initially as NFA can transform into an active corticotroph adenoma.
-
Immunohistochemical marker for ACTH in the resected tumor confirms the diagnosis.
Search for other papers by J Bukowczan in
Google Scholar
PubMed
Search for other papers by K Lois in
Google Scholar
PubMed
Search for other papers by M Mathiopoulou in
Google Scholar
PubMed
Search for other papers by A B Grossman in
Google Scholar
PubMed
Search for other papers by R A James in
Google Scholar
PubMed
Summary
Giant prolactinomas are rare tumours of the pituitary, which typically exceed 40 mm in their largest dimension. Impairment of higher cognitive function has been noted post-operatively after transcranial surgery and as a long-term consequence of the radiotherapy treatment. However, there has been little that is reported on such disturbances in relation to the tumour per se, and to our knowledge, there has been none in terms of responsivity to dopamine agonist therapy and shrinkage in these tumours. We present a case of successful restoration of severely impaired cognitive functions achieved safely after significant adenoma involution with medical treatment alone.
Learning points
-
Giant prolactinomas can be present with profound cognitive defects.
-
Dopamine agonists remain in the mainstay first-line treatment of giant prolactinomas.
-
Mechanisms of the reversible cognitive impairment associated with giant prolactinoma treatment appear to be complex and remain open to further studies.
-
Young patients with giant prolactinomas mandate genetic testing towards familial predisposition.
