Chromophobe Adenoma Manifesting as a Nasopharyngeal Mass Henry Chessin, MD; Nelson Urdaneta, MD; Howard Smith, MD; John Van Gilder, MD
\s=b\ A 64-year-old woman had a sphenoid sinus tumor and nasopharyngeal mass verified to be a nasopharyngeal chromophobe adenoma with no evidence of sellar enlargement or involvement. The possibility that this mass arose from ectopic hypophyseal tissue located in the pharynx is proposed.
(Arch Otolaryngol 102:631-633, 1976)
manifesting as nasal tumors have reported. These latter reported cases involving a nasal or nasopharyn¬ geal lesion were invariably accompa¬ nied by evidence of an intrasellar been
mass
'"'
with sellar destruction. Of inter¬ case report is the finding of
est in this a
a
nasopharyngeal mass, histologically chromophobe adenoma, with no
obvious evidence of intrasellar tu¬
Although
pituitary adenomas often enlarge beyond the confines of the sella turcica, it is unusual for them to extend by way of the pharyngeal pathway that was first described by Jefferson.1 Henderson verified naso¬ pharyngeal extension in eight of 338 patients with pituitary tumors,- and very infrequently, pituitary adenomas .
Accepted
for publication June 18, 1976. From the Departments of Therapeutic Radiology (Drs Chessin and Urdaneta), Otolaryngology (Dr Smith), and Neurosurgery (Dr Van Gilder), Yale University School of Medicine, New Haven, Conn. Reprint requests to Department of Therapeutic Radiology, Yale University School of Medicine, 333 Cedar St, New Haven, CN 06510
(Dr Chessin).
mor.
REPORT OF A CASE A 64-year-old woman was seen in February 1974 with a three-year history of nasal congestion, previously attributed to allergic rhinitis and nasal polyps. On physical examination there was complete nasal obstruction, with pale blue boggy nasal membranes as well as total occlusion of the posterior part of the nasopharynx by a tumor mass. There was no history of headaches or visual loss, and the patient had been examined at intervals since 1971 by her ophthalmologist, who had noted optic atrophy OS with normal visual fields. Polytomography prior to admission showed a mucus cyst in the left maxillary sinus and a large rounded tumor extending anteri¬ orly from the sphenoid sinus with erosion
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
Fig 1.—Left, Preoperative lateral skull polytome demonstrating mass (arrow) in posterior part of nasopharynx. Right, Polytome of pneumoencephalogram one year after surgery and irradiation showing no evidence of suprasellar (A) or intrasphenoid sinus (B) abnormality.
Fig 2.—Transpalatal
view of tumor
prior
to excision.
Fig 3.—Schematic shown in Fig 2.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
diagram
of
surgical
field
of the posterior border of the sphenoid sinus (Fig 1, left). The sella turcica was normal. The patient underwent a transpal¬ atal excision of the lesion, which was continuous anteriorly with the posterior part of the bony septum and posteriorly with the sphenoid sinus. The tumor was excised subtotally with the posterior part of the nasopharyngeal mucosa. Histologie examination showed the specimen to be a chromophobe adenoma. Figure 2 shows the appearance of the mass at the time of surgery. Figure 3 is a schematic drawing of
Fig 2.
patient was referred for radiation therapy following surgery. Neuro-ophthalmologic evaluation at that time disclosed no visual field deficit, but laminograms of The
the sella demonstrated evidence of residual tumor in the sphenoid sinus on the left side. There was demineralization of the left pterygoid plate at its junction with the
sphenoid body, suggesting
tumor exten¬
sion into that bony strut. There was no evidence of erosion or expansion of the floor of the sella turcica. The patient received 5,000 rads during a period of 38 days from a 6 meV linear accelerator through opposed lateral ports that covered the nasopharynx and the pituitary fossa. An additional 1,000 rads during a period of eight days was then given to the area of the nasopharyngeal mass to assure local control. The treatments were administered in May and June 1974 and were tolerated well. In July 1975, the patient was admitted to Yale-New Haven Hospital with the chief complaint of decreasing vision in the left eye of six months' duration. She again denied headaches or visual changes in the right eye. Examination of the nasopharynx and nasal cavity showed no recurrent tumor. Visual acuity was 20/25 OD and 20/ 40 +2 OS. Optic disc pallor with prominent cupping and constriction of visual fields in all quadrants of the left eye was demon¬ strated on Goldmann perimetry. A pneu¬ moencephalogram demonstrated no evi¬ dence of suprasellar mass or impingement on the optic nerves (Fig 1, right). Repeat laminograms of the sella turcica showed no evidence of change when compared with
original x-ray films from 1974. Results of electroencephalogram, brain scan, and spinal fluid examination were all within normal limits. Endocrine evaluation, in¬ cluding morning and evening plasma cortisol levels, thyroid indexes, and 24-hour urinary excretion of 17-hydroxycorticosteroids, was within normal limits. The patient was discharged with no evidence of recurrence found.
COMMENT the hypophysis is
Embryologically,
formed from two separate primordial parts, the Rathke pocket and the infundibular process. The Rathke pocket is an extension of the stomodeal depression. This depression elongates later in development, and its blind end becomes closely applied to the infundibular process. The orig¬ inal stalk narrows and loses continuity with its origin. Patten observes, The commonest evident structural abnor¬ mality of the hypophysis is the presence of ectopie anterior lobe tissue along the course of ingrowth of Rathke's pocket. There are three locations in which these accessory masses of anterior lobe tissue may occur: (1) Within the depression (sella turcica) in the sphenoid bone in which the hypophysis lies, but outside the capsule of the gland, (2) within the substance of the sphenoid bone, and (3) in the soft tissues of the dorsal wall of the pharynx. The last location is by far the most common one... .*
Melchionna and Moore7 in 1938
reported the finding of typical or atypical pituitary tissue in the phar¬ yngeal mucosa in approximately 95% of unselected cases examined. These cells were histologically identical with those in the anterior lobe of the pitui¬ tary gland, and these authors labeled this tissue "pharyngeal pituitary gland." In a postmortem study of 24 female subjects/ pharyngeal hypophyseal tissue was found in all cases, located in the mucoperiosteum of the
nasopharyngeal roof, centered on the posterior vomerosphenoidal articula¬ tion in the median plane. In women 50 years of age or greater, the pharyn¬ geal pituitary tissue tended to be hypertrophie, and it was hypothesized that
as women
age, the sellar adeno-
hypophysis decreases in size and the pharyngeal hypophysis hypertrophies and increases in importance as a of adenohypophyseal hor¬ source mones."
In
our
patient, all the evidence indi¬
cates that there has
intrasellar
mass
or
never
been any
expansion. Al¬
though no surgical confirmation has been obtained, all studies performed tend to support this conclusion. The
possibility of the chromophobe adeno¬ ma having its origin from a deposit of hypophyseal tissue located in the pharynx is likely. It is of interest that this lesion has arisen in an elderly woman, in whom pharyngeal hypo¬ physeal tissue tends to become hypertrophied." The cause of this patient's visual loss and optic atrophy in the left eye remains unclear.
References 1. Jefferson G: Extrasellar extensions of
pitui-
tary adenomas. Proc R Soc Med 33:433-458,
1970. 2. Henderson WR: The pituitary adenomata: A follow-up study of the surgical results in 338 cases (Dr Harvey Cushing's series). Br J Surg 26:811-921, 1938-39. 3. Sadick MK, Nagori MA, Jafarey NA: Chromophobe adenoma of the pituitary gland masquerading as bilateral nasal polypi: Report of a case. J Laryngol Otol 88:169-173, 1974. 4. Shea JJ: Nasal tumors of pituitary origin (report of a case). Trans Am Laryngol Rhinol Otol Soc 47:201-203, 1941. 5. Kay S, Lees JK, Stout AP: Pituitary chromophobe tumors of the nasal cavity. Cancer 3:695-704, 1950. 6. Patten BM: Ductless glands and pharyngeal derivatives, in Human Embryology. Philadelphia, The Blakiston Co, 1948, pp 525-528. 7. Melchionna RH, Moore RA: The pharyngeal pituitary gland. Am J Pathol 14:763-771, 1938. 8. McGrath P: Extrasellar adenohypophyseal tissue in the female. Australas Radiol 14:241-247, 1970.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
\s=b\ A 64-year-old woman had a sphenoid sinus tumor and nasopharyngeal mass verified to be a nasopharyngeal chromophobe adenoma with no evidence of sellar enlargement or involvement. The possibility that this mass arose from ectopic hypophyseal tissue located in the pharynx is proposed.
(Arch Otolaryngol 102:631-633, 1976)
manifesting as nasal tumors have reported. These latter reported cases involving a nasal or nasopharyn¬ geal lesion were invariably accompa¬ nied by evidence of an intrasellar been
mass
'"'
with sellar destruction. Of inter¬ case report is the finding of
est in this a
a
nasopharyngeal mass, histologically chromophobe adenoma, with no
obvious evidence of intrasellar tu¬
Although
pituitary adenomas often enlarge beyond the confines of the sella turcica, it is unusual for them to extend by way of the pharyngeal pathway that was first described by Jefferson.1 Henderson verified naso¬ pharyngeal extension in eight of 338 patients with pituitary tumors,- and very infrequently, pituitary adenomas .
Accepted
for publication June 18, 1976. From the Departments of Therapeutic Radiology (Drs Chessin and Urdaneta), Otolaryngology (Dr Smith), and Neurosurgery (Dr Van Gilder), Yale University School of Medicine, New Haven, Conn. Reprint requests to Department of Therapeutic Radiology, Yale University School of Medicine, 333 Cedar St, New Haven, CN 06510
(Dr Chessin).
mor.
REPORT OF A CASE A 64-year-old woman was seen in February 1974 with a three-year history of nasal congestion, previously attributed to allergic rhinitis and nasal polyps. On physical examination there was complete nasal obstruction, with pale blue boggy nasal membranes as well as total occlusion of the posterior part of the nasopharynx by a tumor mass. There was no history of headaches or visual loss, and the patient had been examined at intervals since 1971 by her ophthalmologist, who had noted optic atrophy OS with normal visual fields. Polytomography prior to admission showed a mucus cyst in the left maxillary sinus and a large rounded tumor extending anteri¬ orly from the sphenoid sinus with erosion
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
Fig 1.—Left, Preoperative lateral skull polytome demonstrating mass (arrow) in posterior part of nasopharynx. Right, Polytome of pneumoencephalogram one year after surgery and irradiation showing no evidence of suprasellar (A) or intrasphenoid sinus (B) abnormality.
Fig 2.—Transpalatal
view of tumor
prior
to excision.
Fig 3.—Schematic shown in Fig 2.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
diagram
of
surgical
field
of the posterior border of the sphenoid sinus (Fig 1, left). The sella turcica was normal. The patient underwent a transpal¬ atal excision of the lesion, which was continuous anteriorly with the posterior part of the bony septum and posteriorly with the sphenoid sinus. The tumor was excised subtotally with the posterior part of the nasopharyngeal mucosa. Histologie examination showed the specimen to be a chromophobe adenoma. Figure 2 shows the appearance of the mass at the time of surgery. Figure 3 is a schematic drawing of
Fig 2.
patient was referred for radiation therapy following surgery. Neuro-ophthalmologic evaluation at that time disclosed no visual field deficit, but laminograms of The
the sella demonstrated evidence of residual tumor in the sphenoid sinus on the left side. There was demineralization of the left pterygoid plate at its junction with the
sphenoid body, suggesting
tumor exten¬
sion into that bony strut. There was no evidence of erosion or expansion of the floor of the sella turcica. The patient received 5,000 rads during a period of 38 days from a 6 meV linear accelerator through opposed lateral ports that covered the nasopharynx and the pituitary fossa. An additional 1,000 rads during a period of eight days was then given to the area of the nasopharyngeal mass to assure local control. The treatments were administered in May and June 1974 and were tolerated well. In July 1975, the patient was admitted to Yale-New Haven Hospital with the chief complaint of decreasing vision in the left eye of six months' duration. She again denied headaches or visual changes in the right eye. Examination of the nasopharynx and nasal cavity showed no recurrent tumor. Visual acuity was 20/25 OD and 20/ 40 +2 OS. Optic disc pallor with prominent cupping and constriction of visual fields in all quadrants of the left eye was demon¬ strated on Goldmann perimetry. A pneu¬ moencephalogram demonstrated no evi¬ dence of suprasellar mass or impingement on the optic nerves (Fig 1, right). Repeat laminograms of the sella turcica showed no evidence of change when compared with
original x-ray films from 1974. Results of electroencephalogram, brain scan, and spinal fluid examination were all within normal limits. Endocrine evaluation, in¬ cluding morning and evening plasma cortisol levels, thyroid indexes, and 24-hour urinary excretion of 17-hydroxycorticosteroids, was within normal limits. The patient was discharged with no evidence of recurrence found.
COMMENT the hypophysis is
Embryologically,
formed from two separate primordial parts, the Rathke pocket and the infundibular process. The Rathke pocket is an extension of the stomodeal depression. This depression elongates later in development, and its blind end becomes closely applied to the infundibular process. The orig¬ inal stalk narrows and loses continuity with its origin. Patten observes, The commonest evident structural abnor¬ mality of the hypophysis is the presence of ectopie anterior lobe tissue along the course of ingrowth of Rathke's pocket. There are three locations in which these accessory masses of anterior lobe tissue may occur: (1) Within the depression (sella turcica) in the sphenoid bone in which the hypophysis lies, but outside the capsule of the gland, (2) within the substance of the sphenoid bone, and (3) in the soft tissues of the dorsal wall of the pharynx. The last location is by far the most common one... .*
Melchionna and Moore7 in 1938
reported the finding of typical or atypical pituitary tissue in the phar¬ yngeal mucosa in approximately 95% of unselected cases examined. These cells were histologically identical with those in the anterior lobe of the pitui¬ tary gland, and these authors labeled this tissue "pharyngeal pituitary gland." In a postmortem study of 24 female subjects/ pharyngeal hypophyseal tissue was found in all cases, located in the mucoperiosteum of the
nasopharyngeal roof, centered on the posterior vomerosphenoidal articula¬ tion in the median plane. In women 50 years of age or greater, the pharyn¬ geal pituitary tissue tended to be hypertrophie, and it was hypothesized that
as women
age, the sellar adeno-
hypophysis decreases in size and the pharyngeal hypophysis hypertrophies and increases in importance as a of adenohypophyseal hor¬ source mones."
In
our
patient, all the evidence indi¬
cates that there has
intrasellar
mass
or
never
been any
expansion. Al¬
though no surgical confirmation has been obtained, all studies performed tend to support this conclusion. The
possibility of the chromophobe adeno¬ ma having its origin from a deposit of hypophyseal tissue located in the pharynx is likely. It is of interest that this lesion has arisen in an elderly woman, in whom pharyngeal hypo¬ physeal tissue tends to become hypertrophied." The cause of this patient's visual loss and optic atrophy in the left eye remains unclear.
References 1. Jefferson G: Extrasellar extensions of
pitui-
tary adenomas. Proc R Soc Med 33:433-458,
1970. 2. Henderson WR: The pituitary adenomata: A follow-up study of the surgical results in 338 cases (Dr Harvey Cushing's series). Br J Surg 26:811-921, 1938-39. 3. Sadick MK, Nagori MA, Jafarey NA: Chromophobe adenoma of the pituitary gland masquerading as bilateral nasal polypi: Report of a case. J Laryngol Otol 88:169-173, 1974. 4. Shea JJ: Nasal tumors of pituitary origin (report of a case). Trans Am Laryngol Rhinol Otol Soc 47:201-203, 1941. 5. Kay S, Lees JK, Stout AP: Pituitary chromophobe tumors of the nasal cavity. Cancer 3:695-704, 1950. 6. Patten BM: Ductless glands and pharyngeal derivatives, in Human Embryology. Philadelphia, The Blakiston Co, 1948, pp 525-528. 7. Melchionna RH, Moore RA: The pharyngeal pituitary gland. Am J Pathol 14:763-771, 1938. 8. McGrath P: Extrasellar adenohypophyseal tissue in the female. Australas Radiol 14:241-247, 1970.
Downloaded From: http://archotol.jamanetwork.com/ by a University of Arizona Health Sciences Library User on 05/28/2015
