0021-972x/92/7404-0811$03.00/0 Journal of Clinical Endocrinology Copyright 0 1992 by The Endocrine
and
Metabolism Society
Vol. 74, No. 4 Printed in U.S.A.
Nasopharyngeal Presentation of Pituitary Differential Diagnosis and Treatment
Tumors.
AART-JAN van der LELY, PAUL P. M. KNEGT, STANLEY Z. STEFANKO, HERVfi L. J. TANGHE, RAM SINGH, AND STEVEN W. J. LAMBERTS Department of Medicine (A.L., S.L.), Otorhinology (P.K.), Pathology (5’S), Neuroradiology Neurosurgery (R.S.), Erasmus University, Rotterdam, The Netherlands
ABSTRACT.
The intranasal presentation of pituitary tumors is rare. We describe six patients with supposedly intranasal carcinomas, treated by surgery, local chemotherapy, and/or radiotherapy. Because of the favorable clinical course, immunohistochemical reexamination of tumor tissue was done, which showed a macroprolactinoma in four and a nonfunctioning pituitary adenoma in two patients. Interestingly, anterior pituitary function was normal in four and only slightly disturbed in two
(H.T.),
and
of them. The radiological appearance of the sellar region was completely normal in two patients. Routine immuno-histochemistry would have prevented inappropriately aggressive therapy. Dopamine agonist therapy was effective in the four macroprolactinoma patients, but the nasopharyngeal localization of the tumor seems to increase the risk of rhinorrhea and/or meningitis. (J Clin Endocrinol Metab 74: 811-813, 1992)
M
ALIGNANT tumors of the paranasal sinuses account for only 3% of all tumors of the upper respiratory tract (1, 2). Squamous cell and undifferentiated carcinoma are the most common (60%), whereas esthesioneuroblastomas are very rare paranasal tumors (1). In the present study we describe four patients with macroprolactinomas and two patients with nonfunctioning pituitary tumors who initially presented with complaints of nasal origin. In the clinical history no anterior pituitary insufficiency was noticed, whereas CT scanning of the sellar region was normal in two.
of the paranasal sinusesand the baseof the skull. Becauseof the excellent condition and the unexpected long survival after 2 yr, the biopsy was reexamined. Immuno-histochemical reexamination with anti-PRL showed a PRL-secreting pituitary tumor. Indeed, serum PRL levels were greatly elevated (Table 1). Endocrine evaluation showedonly a slightly impaired reaction to metyrapone (Table 1). Treatment with bromocriptine (7.5 mg daily) was started. After 6 months rhinorrhea and a life-threatening meningitis occurred. CT examination showed central necrosisaswell asairtrapping in- and around the tumor. Patient
Materials and Methods The metyrapone test (4.5 g/24 h), as well as the TRH (200 pg) and LHRH (100 pg) test have been previously describedin detail (3-5). Circulating hormone levels were measuredwith commercialRIA kits. (T4, TSH, Behring, Marburg, Germany; estradiol, testosterone,deoxycortisol, Diagnostic Products Corporation, Los Angeles, CA; LH, TSH, PRL, GH, Medgenix, Brussels,Belgium). Cross-reactions,inter- and intraassay variations of thesedeterminations have been previously described (6). Immuno-histological examination was done with antisera againstPRL, GH, LH-/3, FSH-/3, and a-subunits.
2
This 61-yr-old woman was treated with surgery and radiotherapy becauseof a supposedly undifferentiated paranasal carcinoma, causing destruction of the paranasal sinusesand the sellar region. One year after treatment the diagnosiswas reconsideredbecauseof her excellent clinical condition and the absenceof tumor progression.Very high circulating PRL levels were found (4200 pg/L). LH, FSH, and estradiol levels were lowered (Table 1). Immuno-histochemicalreexamination of the tumor tissueshowedthe presenceof PRL-containing cells. She was treated successfullywith bromocriptine (5 mg daily) for 8 w
Case Reports
Patient
Patient
1
This 45yr-old male was treated with radiotherapy because of a supposedlyundifferentiated carcinoma,causingdestruction Received March 11, 1991. Address correspondence and requests for reprints to: A. J. van Lely, M.D., Department of Medicine, University Hospital Dijkzigt, Molewaterplein, 3015 GD Rotterdam, The Netherlands.
der 40
3
This 61-yr-old male was admitted becauseof a supposedly esthesioneuroblastoma,causing destruction of the sphenoid, the baseof the skull, and the sellarregion. Immuno-histochemical reexamination showedPRL-containing cells. At that time the patient was in good clinical condition. Circulating PRL were very high (5200 pg/L). Endocrine evaluation showedno
811
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 22 January 2016. at 09:46 For personal use only. No other uses without permission. . All rights reserved.
812
VAN DER LELY
TABLE
JCE & M .1992 Vol74.No4
ET AL.
1. Baseline hormone levels in six patients with a large pituitary tumor with involvement of the pharynx or paranasal sinuses
Pat. no.
Sex (M/F)
Age W
PRL (r/L”)
T4 (nmol/L*)
TSH (mu/L”)
1 M 45 75,000 108 0.81 2 F 61 4,200 132 1.03 3 M 61 5,200 81 0.52 4 M 63 10,700 70 0.80 5 M 50 3.8 85 1.35 6 F 44 6.5 186 1.88 ’ PRL, n < 15 fig/L. * T1, n = 64-132 nmol/L. c TSH, n = 0.2-4.2 mu/L. d LH/FSH, n = l-10 U/L. e Testosterone, n = lo-30 nmol/L. I Estradiol, n = 40-800 pmol/L. B Deoxycortisol after 6 X 750 mg of Metyrapone orally, n > 350 nmol/L. h This patient used a contraconceptive drug, at the time the blood samples were
LH/FSH KJILd) 2.417.3 6.910.7 4.315.2 2.614.6 1.115.4 1.2/1.0h
Testosterone (nmol/L”)
E2 (pmol/W
Metyrapone test (deoxycortisol, nmol/L#)
13.0 23 14.7 13.0 20.9
and
Metabolism Society
Vol. 74, No. 4 Printed in U.S.A.
Nasopharyngeal Presentation of Pituitary Differential Diagnosis and Treatment
Tumors.
AART-JAN van der LELY, PAUL P. M. KNEGT, STANLEY Z. STEFANKO, HERVfi L. J. TANGHE, RAM SINGH, AND STEVEN W. J. LAMBERTS Department of Medicine (A.L., S.L.), Otorhinology (P.K.), Pathology (5’S), Neuroradiology Neurosurgery (R.S.), Erasmus University, Rotterdam, The Netherlands
ABSTRACT.
The intranasal presentation of pituitary tumors is rare. We describe six patients with supposedly intranasal carcinomas, treated by surgery, local chemotherapy, and/or radiotherapy. Because of the favorable clinical course, immunohistochemical reexamination of tumor tissue was done, which showed a macroprolactinoma in four and a nonfunctioning pituitary adenoma in two patients. Interestingly, anterior pituitary function was normal in four and only slightly disturbed in two
(H.T.),
and
of them. The radiological appearance of the sellar region was completely normal in two patients. Routine immuno-histochemistry would have prevented inappropriately aggressive therapy. Dopamine agonist therapy was effective in the four macroprolactinoma patients, but the nasopharyngeal localization of the tumor seems to increase the risk of rhinorrhea and/or meningitis. (J Clin Endocrinol Metab 74: 811-813, 1992)
M
ALIGNANT tumors of the paranasal sinuses account for only 3% of all tumors of the upper respiratory tract (1, 2). Squamous cell and undifferentiated carcinoma are the most common (60%), whereas esthesioneuroblastomas are very rare paranasal tumors (1). In the present study we describe four patients with macroprolactinomas and two patients with nonfunctioning pituitary tumors who initially presented with complaints of nasal origin. In the clinical history no anterior pituitary insufficiency was noticed, whereas CT scanning of the sellar region was normal in two.
of the paranasal sinusesand the baseof the skull. Becauseof the excellent condition and the unexpected long survival after 2 yr, the biopsy was reexamined. Immuno-histochemical reexamination with anti-PRL showed a PRL-secreting pituitary tumor. Indeed, serum PRL levels were greatly elevated (Table 1). Endocrine evaluation showedonly a slightly impaired reaction to metyrapone (Table 1). Treatment with bromocriptine (7.5 mg daily) was started. After 6 months rhinorrhea and a life-threatening meningitis occurred. CT examination showed central necrosisaswell asairtrapping in- and around the tumor. Patient
Materials and Methods The metyrapone test (4.5 g/24 h), as well as the TRH (200 pg) and LHRH (100 pg) test have been previously describedin detail (3-5). Circulating hormone levels were measuredwith commercialRIA kits. (T4, TSH, Behring, Marburg, Germany; estradiol, testosterone,deoxycortisol, Diagnostic Products Corporation, Los Angeles, CA; LH, TSH, PRL, GH, Medgenix, Brussels,Belgium). Cross-reactions,inter- and intraassay variations of thesedeterminations have been previously described (6). Immuno-histological examination was done with antisera againstPRL, GH, LH-/3, FSH-/3, and a-subunits.
2
This 61-yr-old woman was treated with surgery and radiotherapy becauseof a supposedly undifferentiated paranasal carcinoma, causing destruction of the paranasal sinusesand the sellar region. One year after treatment the diagnosiswas reconsideredbecauseof her excellent clinical condition and the absenceof tumor progression.Very high circulating PRL levels were found (4200 pg/L). LH, FSH, and estradiol levels were lowered (Table 1). Immuno-histochemicalreexamination of the tumor tissueshowedthe presenceof PRL-containing cells. She was treated successfullywith bromocriptine (5 mg daily) for 8 w
Case Reports
Patient
Patient
1
This 45yr-old male was treated with radiotherapy because of a supposedlyundifferentiated carcinoma,causingdestruction Received March 11, 1991. Address correspondence and requests for reprints to: A. J. van Lely, M.D., Department of Medicine, University Hospital Dijkzigt, Molewaterplein, 3015 GD Rotterdam, The Netherlands.
der 40
3
This 61-yr-old male was admitted becauseof a supposedly esthesioneuroblastoma,causing destruction of the sphenoid, the baseof the skull, and the sellarregion. Immuno-histochemical reexamination showedPRL-containing cells. At that time the patient was in good clinical condition. Circulating PRL were very high (5200 pg/L). Endocrine evaluation showedno
811
The Endocrine Society. Downloaded from press.endocrine.org by [${individualUser.displayName}] on 22 January 2016. at 09:46 For personal use only. No other uses without permission. . All rights reserved.
812
VAN DER LELY
TABLE
JCE & M .1992 Vol74.No4
ET AL.
1. Baseline hormone levels in six patients with a large pituitary tumor with involvement of the pharynx or paranasal sinuses
Pat. no.
Sex (M/F)
Age W
PRL (r/L”)
T4 (nmol/L*)
TSH (mu/L”)
1 M 45 75,000 108 0.81 2 F 61 4,200 132 1.03 3 M 61 5,200 81 0.52 4 M 63 10,700 70 0.80 5 M 50 3.8 85 1.35 6 F 44 6.5 186 1.88 ’ PRL, n < 15 fig/L. * T1, n = 64-132 nmol/L. c TSH, n = 0.2-4.2 mu/L. d LH/FSH, n = l-10 U/L. e Testosterone, n = lo-30 nmol/L. I Estradiol, n = 40-800 pmol/L. B Deoxycortisol after 6 X 750 mg of Metyrapone orally, n > 350 nmol/L. h This patient used a contraconceptive drug, at the time the blood samples were
LH/FSH KJILd) 2.417.3 6.910.7 4.315.2 2.614.6 1.115.4 1.2/1.0h
Testosterone (nmol/L”)
E2 (pmol/W
Metyrapone test (deoxycortisol, nmol/L#)
13.0 23 14.7 13.0 20.9
