n March 2015, a 62-year-old woman was admitted to our hospital.
She complained of progressive visual disturbance, which began about 4 years ago and was treated as cataract in local hospital, but no relief was seen.
On the contrary, the symptoms aggravated half a year ago, together with headache, left eye pain, tearing and increased secretions, and the computed tomography (CT) scan of the brain in local hospital showed a sellar region lesion.
Besides, 2 years earlier, the patient underwent resection of melanoma in the left heel (T2N0M0, ki67 3–5%, Stage II), followed by resection of the recurred melanoma nearby the primary site 15 months later (T3N3M0, Stage III), without lymphadenectomy.
She had no family history of melanoma.
On physical examination, the patient had bilateral temporal hemianopsia, the right finger counting was 1 m, and the left finger counting was no more than 0.5 m.
Enlarged lymph nodes were palpable in the right groin.
On ophthalmologic examination, the patient had right vision of 0.4 and left vision of 0.08, with the same intraocular pressure 15 mm Hg bilaterally.
The optometry found the right eye of +6.00DS/+0.25DC∗65° and the left eye of +6.25DS/+0.50DC∗20°.
The patient had maculopathy of both eyes and optic atrophy of the left eye.
Light reflex and eye movement of both eyes were normal.
CT scans of the brain parenchyma, orbital, and chest were unremarkable.
CT scan and ultrasound examination of the abdomen showed hepatic portal and retroperitoneal lymphadenectasis and enlarged left lobe of the liver with substantial placeholder lesions.
Ultrasound examination of bilateral inguinal lymph nodes discovered multiple low echo light groups, the largest of which was 31 mm in diameter, with hilus of the echo and asymmetrical thickening of the skin.
CT scan of sellar region revealed a crumby mass, protruding out of the sphenoid sinus, with obscure boundary and bone destruction.
And the average CT value of the mass was 46 HU.
Sellar region magnetic resonance imaging (MRI) revealed a round mass of 30 mm in diameter in the enlarged sellae (Fig.1A, B).
The mass showed isointense in T1-weighted images (T1-WI) and T2-weighted images (T2-WI), with homogeneous enhancement after Gadolinium-DTPA injection, and dural tail sign was seen.
Small foci inside the tumor showed hyperintense signals in T1-WI and hypointense signals in T2-WI, without enhancement.
And it was seen that the mass penetrated meninges, surrounded the left internal carotid artery, and was blurred with the left optic nerve.
Pituitary stalk became shorter with a right displacement.
Laboratory findings revealed increased levels of prolactin (119.08 μg/L, normal range 5.99–30.04 μg/L) and cortisol (677.10 nmol/L, normal range 118.60–618.00 nmol/L) and decreased levels of free thyroxine (FT4) (6.04 pmol/L, normal range 12.00–22.00 pmol/L) and free triiodothyronine (FT3) (2.09 pmol/L, normal range 3.50–6.50 pmol/L).
The patient was diagnosed with a giant prolactinoma.
The patient underwent transnasal transsphenoidal surgery to remove the tumor and relieve the compression of the optic nerve.
Intraoperatively, it was seen that the tumor invaded and filled the left interval of the sphenoid sinus, and part of bone in sellar floor and left side parasellar was destroyed and absorbed.
A little normal pituitary tissue was seen in the top right of tumor in the sellar turcica.
The tumor was reddish black with extremely rich blood supply and had close adhesion to the surrounding structure.
The texture in the center of the tumor was soft and much tougher over the rim.
Intraoperative frozen-section examination found melanin granules, and it was considered to be malignant melanoma or meningioma.
The tumor cells were composed of eosinophilic staining epithelial cells.
Most of cell nuclei were round, a few were reniform and hippocrepiform with evident nucleoli, and nuclear fission was seen.
The tumor showed no evidence of necrosis (Fig.2).
The tumor was immunopositive focally for melanoma-specific markers such as S-100, HMB45, and Vimentin, and immunopositive for neuroendocrine tumor markers such as CgA and Syn (Fig.3).
The Ki67 index was 3% to 5%; it was higher in metastatic melanoma than in the adenomatous component.
Taken the melanoma history and suspected lymph node and hepatic metastasis into consideration, the patient was diagnosised with MMPA.
After surgery, significant relief was seen in visual field and headache, and the level of prolactin, cortisol, and FT4 returned to normal with hormone replacement therapy.
Because the focal liver lesions and lymphadenectasis did not cause much discomfort, the patient refused any further surgical intervention or other treatment.
She was discharged from the hospital immediately and was disease free until 2 months after the third surgery.
The patient successively found new melanoma metastatic sites in the skin of lower left leg, knees, the upper left leg, the left groin and the right groin, and the right leg.
At the follow-up in late January 2016, the patient could not walk and live by herself and was depressed.
At the latest follow-up, in late January, 2017, the patient was alive with worse symptoms, she had sensory deficits of both legs, which could not move, hyperalgesia of hands and mouth, impaired intelligence, but she lived well with the disease by careful nursing of her daughter.