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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 6 | Page : 1870-1872
Pituicytoma in a Young Male and Review of Literature
Mayur Parkhi1, Kirti Gupta1, Sivashanmugam Dhandapani2, Pravin Salunke2
1 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Submission||19-Nov-2019|
|Date of Decision||01-Dec-2019|
|Date of Acceptance||18-Aug-2020|
|Date of Web Publication||23-Dec-2021|
Dr. Kirti Gupta
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Parkhi M, Gupta K, Dhandapani S, Salunke P. Pituicytoma in a Young Male and Review of Literature. Neurol India 2021;69:1870-2
A 28-year-old gentleman presented with headache, vomiting, and visual blurring of 15 days duration associated with somnolence. Post-gadolinium MRI brain revealed an enhancing sellar-suprasellar mass extending into the third ventricle causing hydrocephalus [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d. The lesion was excised endoscopically through the transsphenoidal route and intraoperative frozen consultation suggested a possibility of meningioma. On histology, the tumor comprised moderately cellular fragments featuring compact sheets of numerous bipolar spindle-shaped cells arranged in fascicular and storiform patterns [Figure 2]a and [Figure 2]b. The tumor cells contained abundant eosinophilic cytoplasm with moderately sized nuclei that were oval to elongated with mild irregularity [Figure 2]c and [Figure 2]d. No mitotic figures, endovascular proliferation, or necrosis were evident. These tumor cells displayed diffuse, strong expression for vimentin, S100, thyroid transcription factor 1 (TTF1) [Figure 3]a,[Figure 3]b,[Figure 3]c but were negative for synaptophysin, EMA, CD34, Bcl 2, and keratin [Figure 3]d. Thus, a diagnosis of pituicytoma (PT) was given. The tumor was not invading the sphenoid mucosa or the dura mater. Postoperatively, the patient had an intraventricular bleed and succumbed later to possible hypothalamic dysfunction.
|Figure 1: (a) T2 sagittal MRI showing almost homogeneous iso to hyperintense sellar-suprasellar mass; (b) Coronal T1 MRI showing the extent of isointense mass in the third ventricle with hydrocephalus; (c and d) sagittal and coronal post-contrast MRI showing homogenous enhancement of the mass|
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|Figure 2: (a-d): Tumor composed of interlacing fascicles and focal storiform arrangement (H and E, original magnification ×100); (b): fascicles composed of monomorphic, bipolar spindle cells (H and E, original magnification × 200); (c): high magnification showing angulated spindle cells (H and E, original magnification × 400); (d): focal areas showing oval to plump spindle cells with little or no atypia. No mitoses were identified (H and E, original magnification ×400).|
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|Figure 3: (a-d): Neoplastic cells immunoreactive for Vimentin (a, ×200), S-100 (b, ×400), TTF-1 (c, ×400) while were negative for synaptophysin, EMA, CD34, Bcl 2, and keratin (d, × 100)|
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PTs are unusual, sellar, and/or suprasellar tumors originating from the pituicytes of the neurohypophysis. These tumors, first described in 1955 by Scothorne, were later elaborated by Brat et al. in 2000 in a case series of nine cases. Following which PT was recognized as a distinct entity in the WHO classification of central nervous system tumors and endocrine tumors., The global incidence rate is unknown due to its low frequency and less published data in the literature. Patients present with clinical symptoms mostly in relation to local mass effects which depends upon the tumor size and location. PTs are typically solid and circumscribed mass lesions of the sellar and suprasellar spaces. On imaging, PTs closely mimic sellar masses like pituitary adenoma, craniopharyngioma or meningioma, and thus are more likely to be misdiagnosed if it depends exclusively on radiological impression. As the tumors are of low grade, the majority of them have a favorable outcome. Presently, the option of treatment is surgical resection as these are slow-growing and localized. Due to its slow-growing nature, recurrence is less likely unless it is incompletely resected. However, still, there is a dilemma regarding the diagnostic and therapeutic approaches to this tumor due to its low frequency and scarcity of larger study cohorts in the literature.
On a thorough literature search, we were able to retrieve a total of 140 cases reported until now [Table 1].,,,,,, The worldwide incidence rate of PT is unknown; however, it represented 0.45% of all non-adenomatous lesions and 0.07% of all sellar lesions. It commonly arises in adults with a median age of 47 years (range, 7–83 years) but has no apparent gender preference (M: E ratio, 1:1). Like most pituitary adenomas, the most common presentation was visual disturbances. The prevalence of hypopituitarism and headache is probably higher. There were seven cases that showed association with Cushing's disease and all were in remission. Our patient presented with visual symptoms, headache, and vomiting for the last two weeks which could mainly be attributed to mass effects. It is difficult to suspect or establish a diagnosis of PTs on radio-imaging due to a lack of any pathognomonic characteristic findings. On imaging, in the majority, the tumor appears solid and well defined while in some it may present as solid-cystic mass. In the literature (n = 132), most of these tumors were sellar-suprasellar (n = 57) in location followed by sellar (n = 39) and suprasellar (n = 33) spaces. In some cases (n = 6), the mass also extends to involve the cavernous sinus. In the present case, the post-gadolinium MRI brain revealed an enhancing sellar-suprasellar mass extending into the third ventricle causing hydrocephalus.
|Table 1: The summary of pituicytomas published in the literature,,,,,,|
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Surgical resection is the preferred treatment for PT, with an extremely low recurrence rate (4.3%) following complete resection. The data about treatment were available in 101 cases; all were surgically treated, 10 of them received postoperative radiotherapy and two cases underwent preoperative embolization of arterial feeders of the tumor. In the majority of cases, the degree of tumor removal was variable and included gross total resection (GTR), subtotal resection, partial resection, or biopsy. GTR is the recommended and most accepted approach, especially when the tumor is well demarcated and soft in consistency. Following GTR, the whole tissue is available for making a more precise diagnosis of PT on histopathology. The postoperative complication was evident in 68 patients, commonly seen were hypopituitarism (60.29%), bleeding (50%), and diabetes insipidus (30.88%) [Table 1]. Among 108 cases, recurrence and persistence of the disease was documented in 6.6% (n = 7) and 38.67% (n = 41), respectively. Neither a single case of malignant transformation nor metastases from a PT has been reported, which reflects its low-grade nature and benign behavior. Follow-up period ranged from 3 to 132 months (n = 96; median, 24 months). Our patient succumbed to death possibly due to postoperative hypothalamic dysfunction.
PTs lack definitive cytomorphological features, hence, they are often misdiagnosed as meningiomas on the intraoperative frozen section, as happened in the present case. However, their morphological features are apparent in formalin-fixed paraffin-embedded sections. The bipolar, fusiform tumoral cells distributed in a compact, fascicular, or storiform pattern in a background of the reticulin-rich stroma is characteristic. Furthermore, immunopositivity for vimentin, S-100, and TTF-1, and negativity for hypophyseal hormones, and neuronal or neuroendocrine markers is characteristic. Positivity for TTF-1 brings in spindle cell oncocytomas, granular cell tumors, and sellar ependymoma among the differentials. The genetic profile of these tumors is largely unknown. In conclusion, we would like to highlight the morphological features of this less-common sellar tumor. Lack of awareness might lead to erroneous diagnoses, for which the patient may be subjected to unnecessary investigations. An appropriate immunohistochemical panel is useful in arriving at the correct diagnosis and should be employed to supplement the characteristic morphological features.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]