Primary sellar melanoma simulating hemorrhagic pituitary adenoma: MR and pathologic findings.
Phylliss M. Chappell, W M Kelly, Mark Ercius
Abstract
Phylliss M. Chappell, W M Kelly, Mark Ercius
Abstract
·Primary intracranial melanoma is uncommon, and primary malignant melanoma of the sella turcica is exceedingly rare. We describe a 35-year-old woman who presented with en docrine dysfunction and CT findings suggestive of a prolactin secreting macroadenoma. Subsequent MR findings revealed homogeneously short T1 and T2 relaxation times, yielding uniformly bright signal on T1 -weighted images and dark signal on T2-weighted images. At high field strength (1 .5 T) , these characteristics seemed to implicate either paramagnetic ef fects suggestive of subacute hemorrhage or the intrinsically short relaxation times of lipidlike or mucinous material that may accumulate in a dermoid or Rathke cleft cyst. Following surgery, a final pathologic review revealed deeply pigmented, nonhemorrhagic malignant melanoma. The paramagnetic ef fect of stable free radicles of melanin is thought to account for the confusing MR characteristics of this uncommon lesion encountered at an unusual location [1-3). Case Report A 35-year-old woman presented with a 3-year history of oligomen orrhea, intermittent galactorrhea, and headache. Her prolactin level was 102.5 ngjml (normal range, 0-20 ngjml). Contrast-enhanced CT revealed a homogeneously hyperden se lesion in the sella turcica with suprasellar extension. High-field-strength MR (G E 1.5 T) confirmed the presence of a pituitary mass, demonstrating very high signal intensity on T1-weighted images, and low sig nal intensity on T2weighted images (Fig. 1 ). The CT and MR appearance of the mass suggested a histologically complex lesion harboring a paramagnetic substance. The combination of clini cal findings, laboratory-docu mented hormonal derangements, and imaging results prompted con sideration of hemorrhagic pituitary adenoma (the favored preoperative diagnosis) or a developmental inclusion tumor of dermal origin . At transphenoidal hypophysectomy, a firm , dark black/purple mass was exposed and a biopsy was done. Frozen section specimens were interpreted as showing no evidence of tumor. Initially , the lesion was thought to represent an area of old hemorrhage with hemosiderin deposition. However, permanent histologic sections demonstrated a markedly pleomorphic tumor. Large amounts of melanin pigment were identified on both Fontana and melanin bleach stains . Prussian blue stain s were negati ve for iron (Fig. 2) . These distinctive histologic findings confirmed the final pathologic diagnosis as malignant mela noma. Postoperatively, ophthalmoscopic, gynecologic, and dermatologic examinations were negative. Endoscopy of the gastrointestinal tract also failed to reveal an extracranial primary tumor. Complete hypo physectomy, followed by moderate-dose radiotherapy was recom mended, but the patient refused further treatment. MR of the sella 3 months later showed regrowth of the tumor, filling the entire sella turcica and adjacent suprasellar cistern . Hyperintensity was again noted on T1-weighted images and hypointensity was seen on T2weighted images.
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·Primary intracranial melanoma is uncommon, and primary malignant melanoma of the sella turcica is exceedingly rare. We describe a 35-year-old woman who presented with en docrine dysfunction and CT findings suggestive of a prolactin secreting macroadenoma. Subsequent MR findings revealed homogeneously short T1 and T2 relaxation times, yielding uniformly bright signal on T1 -weighted images and dark signal on T2-weighted images. At high field strength (1 .5 T) , these characteristics seemed to implicate either paramagnetic ef fects suggestive of subacute hemorrhage or the intrinsically short relaxation times of lipidlike or mucinous material that may accumulate in a dermoid or Rathke cleft cyst. Following surgery, a final pathologic review revealed deeply pigmented, nonhemorrhagic malignant melanoma. The paramagnetic ef fect of stable free radicles of melanin is thought to account for the confusing MR characteristics of this uncommon lesion encountered at an unusual location [1-3). Case Report A 35-year-old woman presented with a 3-year history of oligomen orrhea, intermittent galactorrhea, and headache. Her prolactin level was 102.5 ngjml (normal range, 0-20 ngjml). Contrast-enhanced CT revealed a homogeneously hyperden se lesion in the sella turcica with suprasellar extension. High-field-strength MR (G E 1.5 T) confirmed the presence of a pituitary mass, demonstrating very high signal intensity on T1-weighted images, and low sig nal intensity on T2weighted images (Fig. 1 ). The CT and MR appearance of the mass suggested a histologically complex lesion harboring a paramagnetic substance. The combination of clini cal findings, laboratory-docu mented hormonal derangements, and imaging results prompted con sideration of hemorrhagic pituitary adenoma (the favored preoperative diagnosis) or a developmental inclusion tumor of dermal origin . At transphenoidal hypophysectomy, a firm , dark black/purple mass was exposed and a biopsy was done. Frozen section specimens were interpreted as showing no evidence of tumor. Initially , the lesion was thought to represent an area of old hemorrhage with hemosiderin deposition. However, permanent histologic sections demonstrated a markedly pleomorphic tumor. Large amounts of melanin pigment were identified on both Fontana and melanin bleach stains . Prussian blue stain s were negati ve for iron (Fig. 2) . These distinctive histologic findings confirmed the final pathologic diagnosis as malignant mela noma. Postoperatively, ophthalmoscopic, gynecologic, and dermatologic examinations were negative. Endoscopy of the gastrointestinal tract also failed to reveal an extracranial primary tumor. Complete hypo physectomy, followed by moderate-dose radiotherapy was recom mended, but the patient refused further treatment. MR of the sella 3 months later showed regrowth of the tumor, filling the entire sella turcica and adjacent suprasellar cistern . Hyperintensity was again noted on T1-weighted images and hypointensity was seen on T2weighted images.
Key concepts: Sella turcica, Medicine, Pituitary adenoma, Lesion, Pathology, Pituitary gland, Pituitary neoplasm, Radiology