A Letter

Pheochromocytoma and Von Recklinghausen Disease Revealed by Adrenal Incidentaloma  

M. A Amani , N Medjadi , B Benlazaar
Department of Endocrinology and Diabetology 1st November Hospital Oran, Algeria
Author    Correspondence author
International Journal of Clinical Case Reports, 2016, Vol. 6, No. 19   doi: 10.5376/ijccr.2016.06.0019
Received: 22 Feb., 2016    Accepted: 25 Mar., 2016    Published: 08 Aug., 2016
© 2016 BioPublisher Publishing Platform
This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:

Amani M.A., Medjadi N., and Benlazaar B., 2016, Pheochromocytoma and Von Recklinghausen Disease Revealed by Adrenal Incidentaloma, International Journal of Clinical Case Report, 6(19): 1-3 (doi: 10.5376/ijccr.2016.06.0019)

Abstract

Adrenal incidentalomas are discovered incidentally during a radiological examination of the abdomen whatever the reason justifying this exploration.

 

We report the case of a 37 years old woman with no particular family history and who consulted for a right adrenal incidentaloma discovered during an abdominal ultrasound performed for vomiting. Clinical examination revealed a blood pressure-110/70 mmHg, a Menard triad (headaches, palpitations, sweating), a multiheteronodular goiter without signs of thyroid dysfunction, café-au-lait spots and cutaneous neurofibromas and diffuses lentiginous.

 

Laboratory tests confirmed the secretory nature of the mass. A radiological assessment with an abdominopelvic computed tomography (CT) scan showed a right adrenal tissue mass measuring 47×48×38 mm. The MIBG scintigraphy confirmed the neuroendocrine nature of the mass and the absence of other sites.

Keywords
Adrenal incidentaloma; Pheochromocytoma; Von Recklinghausen disease; Adrenalectomy

1 Introduction

Adrenal incidentalomas are discovered incidentally during a radiological examination of the abdomen whatever the reason justifying this exploration (Tabuchi et al., 2015). Furthermore pheochromocytoma, the etiologies of these incidentalomas are various (Tabuchi et al., 2015). Pheochromocytomas are neuroendocrine tumors developed at the expense of cells derived from neural crest. Pheochromocytomas can be sporadic or familial and then be isolated or integrated into multiple endocrine neoplasia (MEN type 1 and 2), phacomatosis (von Recklinghausen disease) or von Hippel Lindau disease (VHL).

 

2 Observation

We report the case of a 37 years old woman with no particular family history and who consulted for a right adrenal incidentaloma discovered during an abdominal ultrasound performed for vomiting. Clinical examination revealed a blood pressure-110/70 mmHg, a Menard triad (headaches, palpitations, sweating), a multiheteronodular goiter without signs of thyroid dysfunction, café-au-lait spots and diffuses lentiginous and cutaneous neurofibromas (Figure 1Figure 2Figure 3).

 

  

Figure 1 Cutaneous lesions: café-au-lait spots 

 

  

Figure 2 Cutaneous lesions: neurofibromas

 

  

Figure 3 Cutaneous lesions: lentiginous

 

Laboratory tests showed: fasting glucose: 0.93 g/L, sodium: 139.1 mmol/L, potassium: 3.80mmol/L, cortisol after 1 mg overnight dexamethasone suppression test: 1.27 μg/dl (negative), elevated urinary derivatives methoxylated: normetanephrine=16.98 mmol/24 hours (>4N) and metanephrine=22.26 mmol/24 hours (>4N), TSH=1.83 μUI/ml, PTH=53.2 pg/ml, calcitonin=11 pg/ml. A radiological assessment with an abdominopelvic computed tomography (CT) scan showed a right adrenal tissue mass measuring 47×48×38 mm (enhanced CT also showed 83% at rate of absolute percentage wash out, and 64% at rate of relative percentage wash out, early density 167 UH, delayed density 60 UH, spontaneous density 39 UH) (Figure 4). The MIBG scintigraphy confirmed the neuroendocrine nature of the mass and the absence of other sites. A right adrenalectomy with subcostal incision was performed and the histological examination revealed a benign pheochromocytoma.

 

  

Figure 4 Abdominopelvic computed tomography

 

3 Discussion

Our patient had a pheochromocytoma associated with von Recklinghausen disease which was revealed by an adrenal incidentaloma. Pheochromocytomas are neuroendocrine tumors developed at the expense of cells derived from neural crest and they are sporadic or familial and then isolated or integrated into multiple endocrine neoplasia (MEN type 1 and 2), phacomatosis (von Recklinghausen disease) or von Hippel Lindau disease (VHL).

 

The etiologies of the adrenal incidentalomas are multiple and their rates are 4.7% (Tabuchi et al., 2015) and 11.68% (Li et al., 2015).

 

The time risk of developing pheochromocytoma is 48-54 months (Loh et al., 2015). The incidence of pheochromocytoma is 0.4% (Loh et al., 2015). Otherwise, the tumor size exceeding 5cm is a criterion for a higher risk of malignancy (Lenderz et al., 2005). In his study, Azzoug S reported 10% of cases of malignant pheochromocytomas (Azzoug et al., 2015). Despite the benign character of the tumor, a long term follow- up is necessary (Haddam et al., 2015).

 

Otherwise, Tancić-Gajić reported the case of a female, 56 years old, followed for an adrenal incidentaloma with MEN type 1 undiagnosed, and which the pheochromocytoma was undetected with a not confirmed diagnosis at biology despite a suggestive clinical picture (Tancić-Gajić et al., 2008). A MRI in favour of an adrenal pheochromocytoma and a positive MIBG scintigraphy led to surgery. Histology revealed a left adrenal pheochromocytoma.

 

4 Conclusion

The management of pheochromocytoma is multidisciplinary. Properly operated, the pheochromocytoma is radically and permanently cured if it is benign and sporadic.

 

Author’s contributions

By signing this letter each of us acknowledges that she or he participated sufficiently in the work to take public responsibility for it. We also declare that there is no conflict of interest that would prejudice the impartiality of this scientific work.

 

References

Azzoug S., Amirou Al., Rabehi L., and Chentli F., 2015, Malignant Pheochromocytoma, International Journal of Clinical Case Reports, 5(21): 1-4

http://dx.doi.org/10.5376/ijccr.2015.05.0021

 

Haddam A.E.M., Fedala S.N., Chentli F., and Meskine D., 2015, Malignant Pheochromocytoma: about Two New Cases, International Journal of Clinical Case Reports, 5(31): 1-5

http://dx.doi.org/10.5376/ijccr.2015.05.0031

 

Lenders J.W.M., Eisenhofer G., Mannelli M., and Pacak K., 2005, Pheochromocytoma, The Lancet, 366: 665-675

http://dx.doi.org/10.1016/S0140-6736(05)67139-5

 

Li L., Dou J., Gu W., Yang G., Du J., Yang L., Zang L., Wang X., Jin N., Ou-Yang J., Lü Z., Ba J., Mu Y., Lu J., Li J., and Pan C., 2014, Etiologies of 1 173 hospitalized cases with adrenal incidentaloma, Zhonghua Yi Xue Za Zhi, 94(8): 587-590

 

Loh H.H., Yee A., Loh H.S., Sukor N., and Kamaruddin N.A., 2015, The natural progression and outcomes of adrenal incidentaloma: a systematic review and meta-analysis, Minerva Endocrinol.

 

Tabuchi Y., Otsuki M., Kasayama S., Kosugi K., Hashimoto K., Yamamoto T., Tsugawa M., Mineo I., Yamada Y., Kurebayashi S., Ohashi M., Umayahara Y., Kouhara H., Nakamura T., Matsuoka T.A., Imagawa A., Funahashi T., and Shimomura I., 2015, Clinical and endocrinological characteristics of adrenal incidentaloma in Osaka region, Japan, Endocr J.

 

Tancić-Gajić M., Vujović S., Tatić S., Stojanović M., Ivović M., and Drezgić M., 2008, Adrenal incidentaloma in neurofibromatosis type 1. Srp Arh Celok Lek., 136(5-6): 295-298

http://dx.doi.org/10.2298/SARH0806295T

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