Recurrent Cushing's disease due to postoperative seeding of tumor cells in the sphenoid sinus: a case report with review of the literature.
This case illustrates that recurrent Cushing's disease can arise from tumor cell seeding into the sphenoid sinus during surgery, highlighting the importance of careful surgical techniques.
Where it sits
this study against the rest of the pasireotide corpusSummary and findings
This case report describes a 30-year-old woman who experienced recurrent Cushing's disease after initial endocrine remission following gross total resection of an ACTH-secreting PitNET. Despite long-term medical therapy including pasireotide, her hypercortisolism progressed until a reoperation revealed a recurrent tumor in the sphenoid sinus. Complete resection of this tumor led to immediate endocrine remission.
Abstract
Recurrent Cushing's disease is most commonly caused by residual or newly developed pituitary neuroendocrine tumor (PitNET) tissue within the sellar region. Extrasellar recurrence due to postoperative tumor cell seeding is exceedingly rare. We report the unique case of a 30-year-old woman who achieved endocrine remission after gross total resection of an ACTH-secreting PitNET but later developed biochemical and clinical recurrence without radiological evidence of a sellar lesion. Despite long-term medical therapy including cabergoline, metyrapone, and pasireotide, the hypercortisolism gradually progressed. Nearly 10 years after the initial surgery, a reoperation was performed to explore the occult tumor. Intraoperatively, a small soft tumor attached to the posterior surface of the anterior wall of the sphenoid sinus was discovered. Histopathological examination confirmed the presence of a recurrent corticotrophic PitNET. A retrospective review of CT, MRI, and octreotide scintigraphy revealed a subtle lesion that had previously been overlooked. Complete resection resulted in an immediate endocrine remission. This case demonstrates that recurrent Cushing's disease may, although rarely, arise from the implantation of tumor cells that drop into the sphenoid sinus during transsphenoidal surgery. Awareness of this mechanism is crucial for evaluating MRI-negative recurrences. Careful inspection of the sphenoid sinus and meticulous irrigation during surgery may help prevent iatrogenic seeding and improve long-term outcomes.
Background
This paper addresses the rare occurrence of recurrent Cushing's disease due to postoperative seeding of tumor cells. Prior knowledge indicates that recurrence is typically linked to residual tumor tissue within the sellar region. Understanding this mechanism is important for clinicians evaluating patients with MRI-negative recurrences.
Methods
This is a case report detailing the clinical course of a single patient. The patient underwent gross total resection of an ACTH-secreting PitNET, followed by long-term medical therapy. The timeline of events spans nearly 10 years, culminating in a reoperation to explore an occult tumor.
Results
The reoperation revealed a small soft tumor in the sphenoid sinus, which was confirmed to be a recurrent corticotrophic PitNET through histopathological examination. Complete resection of this tumor resulted in immediate endocrine remission.
Interpretation
This case highlights a unique mechanism of recurrence that may not be well recognized in clinical practice. While the findings are significant, they are based on a single case, which limits the ability to generalize the results. The small size of the tumor and the specific circumstances of this case may not reflect broader trends in recurrent Cushing's disease.
Key findings
- Reoperation revealed a small soft tumor in the sphenoid sinus.
- Histopathological examination confirmed recurrent corticotrophic PitNET.
- Complete resection resulted in immediate endocrine remission.
Limitations
- Single case report limits generalizability.
- Long-term outcomes of pasireotide therapy not detailed.
- No quantitative data on hormonal levels or tumor size.
- Lack of control group for comparison.