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Review
, 6 (1), 75-100

Ectopic Sphenoid Sinus Pituitary Adenoma (ESSPA) With Normal Anterior Pituitary Gland: A Clinicopathologic and Immunophenotypic Study of 32 Cases With a Comprehensive Review of the English Literature

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Review

Ectopic Sphenoid Sinus Pituitary Adenoma (ESSPA) With Normal Anterior Pituitary Gland: A Clinicopathologic and Immunophenotypic Study of 32 Cases With a Comprehensive Review of the English Literature

Lester D R Thompson et al. Head Neck Pathol.

Abstract

Ectopic sphenoid sinus pituitary adenoma (ESSPA) may arise from a remnant of Rathke's pouch. These tumors are frequently misdiagnosed as other neuroendocrine or epithelial neoplasms which may develop in this site (olfactory neuroblastoma, neuroendocrine carcinoma, sinonasal undifferentiated carcinoma, paraganglioma, melanoma). Thirty-two patients with ESSPA identified in patients with normal pituitary glands (intact sella turcica) were retrospectively retrieved from the consultation files of the authors' institutions. Clinical records were reviewed with follow-up obtained. An immunohistochemical panel was performed on available material. Sixteen males and 16 females, aged 2-84 years (mean, 57.1 years), presented with chronic sinusitis, headache, obstructive symptoms, and visual field defects, although several were asymptomatic (n = 6). By definition, the tumors were centered within the sphenoid sinus and demonstrated, by imaging studies or intraoperative examination, a normal sella turcica without a concurrent pituitary adenoma. A subset of tumors showed extension into the nasal cavity (n = 5) or nasopharynx (n = 9). Mean tumor size was 3.4 cm. The majority of tumors were beneath an intact respiratory epithelium (n = 22), arranged in many different patterns (solid, packets, organoid, pseudorosette-rosette, pseudopapillary, single file, glandular, trabecular, insular). Bone involvement was frequently seen (n = 21). Secretions were present (n = 16). Necrosis was noted in 8 tumors. The tumors showed a variable cellularity, with polygonal, plasmacytoid, granular, and oncocytic tumor cells. Severe pleomorphism was uncommon (n = 5). A delicate, salt-and-pepper chromatin distribution was seen. In addition, there were intranuclear cytoplasmic inclusions (n = 25) and multinucleated tumor cells (n = 18). Mitotic figures were infrequent, with a mean of 1 per 10 HPFs and a <1% proliferation index (Ki-67). There was a vascularized to sclerotic or calcified stroma. Immunohistochemistry highlighted the endocrine nature of the tumors, with synaptophysin (97%), CD56 (91%), NSE (76%) and chromogranin (71%); while pan-cytokeratin was positive in 79%, frequently with a dot-like Golgi accentuation (50%). Reactivity with pituitary hormones included 48% reactive for 2 or more hormones (plurihormonal), and 33% reactive for a single hormone, with prolactin seen most frequently (59%); 19% of cases were non-reactive. The principle differential diagnosis includes olfactory neuroblastoma, neuroendocrine carcinoma, melanoma, and meningioma. All patients were treated with surgery. No patients died from disease, although one patient died with persistent disease (0.8 months). Surgery is curative in the majority of cases, although recurrence/persistence was seen in 4 patients (13.8%). In conclusion, ESSPAs are rare, affecting middle aged patients with non-specific symptoms, showing characteristic light microscopy and immunohistochemical features of their intrasellar counterparts. When encountering a tumor within the sphenoid sinus, ectopic pituitary adenoma must be considered, and pertinent imaging, clinical, and immunohistochemical evaluation undertaken to exclude tumors within the differential diagnosis. This will result in accurate classification, helping to prevent the potentially untoward side effects or complications of incorrect therapy.

Figures

Fig. 1
Fig. 1
A diagrammatic representation of the anatomy around the sphenoid sinus (S), immediately above the nasopharynx (N), and immediately below the sella turcica containing the pituitary gland (P). The cavernous sinus (blue), internal carotid arteries (red) and cranial nerve branches (yellow) all have an intimate relationship with the pituitary fossa and sphenoid sinus (Used by special permission of Amirsys, Inc., Salt Lake City, UT, USA)
Fig. 2
Fig. 2
A mass is noted within the right sphenoid sinus, showing intact wings of the sphenoid bone
Fig. 3
Fig. 3
Fragments of tissue may appear polypoid. Left Lesional hemorrhage and fibrosis surround the neoplastic cells. Right Heavy background stromal fibrosis nearly completely obscures the small cords of compressed tumor cells
Fig. 4
Fig. 4
Left An intact, uninvolved respiratory epithelium is subtended by a zone of fibrosis before the neoplastic proliferation is detected. Right Heavy stromal fibrosis compresses the neoplastic cells into small cords and individually infiltrating tumor cells. Note the bone (right)
Fig. 5
Fig. 5
Left Invasion into bone and cartilage was frequently detected. Right The neoplastic cells invade adjacent to minor mucoserous glands. Note the numerous intranuclear cytoplasmic inclusions
Fig. 6
Fig. 6
Left A solid tumor pattern with delicate fibrovascular stroma. Right Fibrovascular stroma with greater collagen deposition. The tumor is moderately cellular with limited pleomorphism
Fig. 7
Fig. 7
Several different patterns of growth are highlighted. Pseudopapillary (left upper), organoid (right upper), trabecular to organoid (left lower) and pseudorosettes (right lower). Several different cytologic appearances are also seen
Fig. 8
Fig. 8
Left Secretions or concretions are noted within the neoplastic proliferation. Right Tumor necrosis was infrequent, showing a comedonecrosis pattern in this tumor
Fig. 9
Fig. 9
Left High tumor cellularity could mimic other tumors of the upper aerodigestive tract. Note the delicate chromatin distribution, with grooves and small nucleoli. Right Intranuclear cytoplasmic inclusions were frequently seen, showing a well-formed membrane-lined inclusion
Fig. 10
Fig. 10
A variety of cytologic appearances were seen, including plasmacytoid (left upper), polygonal (right upper), epithelioid to spindled (left lower) and small cell type (right lower)
Fig. 11
Fig. 11
Left Profound nuclear pleomorphism within this tumor. Right upper Tumor cell multinucleation was a common feature. Right lower Prominent nucleoli are seen in cells that have granular to eosinophilic cytoplasm and interspersed inflammatory cells
Fig. 12
Fig. 12
Cytokeratin could be seen as a diffuse, cytoplasmic reaction of a “fibrous body” type (left) or producing a delicate “dot-like” perinuclear (Golgi) pattern (right)
Fig. 13
Fig. 13
Neuroendocrine markers were positive, showing a variety of different patterns: Strong diffuse cytoplasmic reaction with synaptophysin (note negative surface epithelium; left upper); a Golgi accentuation could also be seen (left lower); Chromogranin showing a granular strong to moderate cytoplasmic reaction (right upper) or a membranous accentuation (right lower)
Fig. 14
Fig. 14
Left CD56 usually produced a strong, diffuse, membranous reaction in the tumor cells. Right NSE showed a variable reaction, although it was usually a granular cytoplasmic reaction
Fig. 15
Fig. 15
Left Prolactin, when detected, usually yielded a strong reaction. Right Isolated tumor cells stained for specific peptides would look like this case of an ACTH reaction

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