Limits...
Idiopathic SIADH in young patients: don't forget the nose

View Article: PubMed Central - PubMed

ABSTRACT

Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine sinonasal cancer associated by many authors to ectopic production of several biologically active substances. We report a case of a 31-year-old male patient who presented with idiopathic syndrome of inappropriate secretion of antidiuretic hormone (SIADH). During diagnostic work-up, a CT scan of the head was performed and an ethmoidal ONB was detected. Endoscopical surgery followed by radiotherapy was carried out. Immediately after surgery natraemia levels normalised. Five years later the patient is disease-free. To our knowledge, 17 cases of SIADH associated to ONB have been published. In nine reports, idiopathic SIADH promptly led to the diagnosis of the sinonasal mass as in our clinical case, however, in many reports, correct diagnosis was accomplished months to years later. In young patients with idiopathic inappropriate antidiuretic hormone secretion, a neuroendocrine malignancy of the sinonasal area must be excluded.

No MeSH data available.


Related in: MedlinePlus

A-B. Coronal and sagittal T2-weighted images. Pre-operative MRI shows a large, well-defined mass expanding the right ethmoid. The mass lesion exhibits intermediate signal on T2-weighted images, due to its high cellularity; there is no evidence of infiltration of the orbital fat and of the fovea ethmoidalis. Inflammatory/obstructive changes are seen in both right frontal and maxillary sinuses due to mass effect on the ostio-meatal unit. Right lamina papyracea was compressed by the mass, but not interrupted; the orbit, anterior cranial fossa and brain were disease-free. C. Endoscopic view of the right nasal fossa: a fairly well circumscribed, multi-lobulated smooth rounded mass, involving the right ethmoid sinus and the region of middle turbinate is showed. Bony destruction was not recognisable, but the mass effect was prominent. D. Post-operative coronal CT image demonstrates the ethmoido-maxillectomy, without evidence of residual/recurrent disease.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC5384315&req=5

Figure 2: A-B. Coronal and sagittal T2-weighted images. Pre-operative MRI shows a large, well-defined mass expanding the right ethmoid. The mass lesion exhibits intermediate signal on T2-weighted images, due to its high cellularity; there is no evidence of infiltration of the orbital fat and of the fovea ethmoidalis. Inflammatory/obstructive changes are seen in both right frontal and maxillary sinuses due to mass effect on the ostio-meatal unit. Right lamina papyracea was compressed by the mass, but not interrupted; the orbit, anterior cranial fossa and brain were disease-free. C. Endoscopic view of the right nasal fossa: a fairly well circumscribed, multi-lobulated smooth rounded mass, involving the right ethmoid sinus and the region of middle turbinate is showed. Bony destruction was not recognisable, but the mass effect was prominent. D. Post-operative coronal CT image demonstrates the ethmoido-maxillectomy, without evidence of residual/recurrent disease.

Mentions: The patient at this point made return to his home country and was admitted to our hospital for therapeutic planning. MR scan of the head and neck was performed (Fig. 2 A-B), results were consistent with the CT data, additional information were precise dimensions (38x16 mm), slight nasal septum and nasolacrimal duct deformation. Endoscopic nasal evaluation was performed (Fig. 2C). According to Kadish grading19, the lesion was classified as stage B. After tumour board discussion, an endoscopic resection of the lesion without lamina cribra resection and duroplasty was performed (Fig. 2D). Serum natraemia levels normalised the day after surgery. Postoperative radiation therapy was administered. The patient underwent regular endoscopic and radiological follow-up and 5 years later is disease-free.


Idiopathic SIADH in young patients: don't forget the nose
A-B. Coronal and sagittal T2-weighted images. Pre-operative MRI shows a large, well-defined mass expanding the right ethmoid. The mass lesion exhibits intermediate signal on T2-weighted images, due to its high cellularity; there is no evidence of infiltration of the orbital fat and of the fovea ethmoidalis. Inflammatory/obstructive changes are seen in both right frontal and maxillary sinuses due to mass effect on the ostio-meatal unit. Right lamina papyracea was compressed by the mass, but not interrupted; the orbit, anterior cranial fossa and brain were disease-free. C. Endoscopic view of the right nasal fossa: a fairly well circumscribed, multi-lobulated smooth rounded mass, involving the right ethmoid sinus and the region of middle turbinate is showed. Bony destruction was not recognisable, but the mass effect was prominent. D. Post-operative coronal CT image demonstrates the ethmoido-maxillectomy, without evidence of residual/recurrent disease.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5384315&req=5

Figure 2: A-B. Coronal and sagittal T2-weighted images. Pre-operative MRI shows a large, well-defined mass expanding the right ethmoid. The mass lesion exhibits intermediate signal on T2-weighted images, due to its high cellularity; there is no evidence of infiltration of the orbital fat and of the fovea ethmoidalis. Inflammatory/obstructive changes are seen in both right frontal and maxillary sinuses due to mass effect on the ostio-meatal unit. Right lamina papyracea was compressed by the mass, but not interrupted; the orbit, anterior cranial fossa and brain were disease-free. C. Endoscopic view of the right nasal fossa: a fairly well circumscribed, multi-lobulated smooth rounded mass, involving the right ethmoid sinus and the region of middle turbinate is showed. Bony destruction was not recognisable, but the mass effect was prominent. D. Post-operative coronal CT image demonstrates the ethmoido-maxillectomy, without evidence of residual/recurrent disease.
Mentions: The patient at this point made return to his home country and was admitted to our hospital for therapeutic planning. MR scan of the head and neck was performed (Fig. 2 A-B), results were consistent with the CT data, additional information were precise dimensions (38x16 mm), slight nasal septum and nasolacrimal duct deformation. Endoscopic nasal evaluation was performed (Fig. 2C). According to Kadish grading19, the lesion was classified as stage B. After tumour board discussion, an endoscopic resection of the lesion without lamina cribra resection and duroplasty was performed (Fig. 2D). Serum natraemia levels normalised the day after surgery. Postoperative radiation therapy was administered. The patient underwent regular endoscopic and radiological follow-up and 5 years later is disease-free.

View Article: PubMed Central - PubMed

ABSTRACT

Olfactory neuroblastoma (ONB) is an uncommon neuroendocrine sinonasal cancer associated by many authors to ectopic production of several biologically active substances. We report a case of a 31-year-old male patient who presented with idiopathic syndrome of inappropriate secretion of antidiuretic hormone (SIADH). During diagnostic work-up, a CT scan of the head was performed and an ethmoidal ONB was detected. Endoscopical surgery followed by radiotherapy was carried out. Immediately after surgery natraemia levels normalised. Five years later the patient is disease-free. To our knowledge, 17 cases of SIADH associated to ONB have been published. In nine reports, idiopathic SIADH promptly led to the diagnosis of the sinonasal mass as in our clinical case, however, in many reports, correct diagnosis was accomplished months to years later. In young patients with idiopathic inappropriate antidiuretic hormone secretion, a neuroendocrine malignancy of the sinonasal area must be excluded.

No MeSH data available.


Related in: MedlinePlus