neurofibromatosis type 1 ( nf1 ) affects approximately 1 in 3500 patients worldwide and is the most common phakomatoses reported . phakomatoses are characterized by cutaneous pigmentary abnormalities and neoplastic growth of neural crest derived cells . nf1 is an autosomal dominant disorder characterized by multiple soft tissue neurofibromas , caf au lait macules and lisch nodules . nf1 patients have higher predilection to develop tumors like pheochromocytoma , paraganglioma , gastrointestinal stromal tumor , and pancreatic neuroendocrine tumor . most of the pheochromocytomas occur sporadically but familial predisposition is seen in cases of multiple endocrine neoplasia 2 ( men2 ) , nf1 and von hippel - lindau ( vhl ) disease . pheochromocytoma is rarely reported from our country and there are few publications involving small case series.[24 ] ganesh et al . reported a series of 11 pediatric pheochromocytoma patients , where as agarwal et al . reported a series of 93 patients over 15 years . we recently encountered an elderly lady who had prolonged history of neurofibromas and poorly controlled hypertension due to unilateral pheochromocytoma . literature search revealed a single case report from india about the occurrence of pheochromocytoma in a patient with malignant peripheral nerve sheath tumor without nf1 . pheochromocytoma with neurofibromatosis has not been reported earlier from india and hence we report the same in this article . the pain was localized to right lumbar region with dull aching in character and no specific exacerbating or relieving factors . past medical history revealed that she noticed multiple nodular swellings on her body and face for past 50 years . family history revealed similar nodular lesions in her father and sister with no major illness in them or other family members . she was diagnosed to have hypertension 8 years back and is currently requiring more than three antihypertensive drugs for optimal control . examination showed blood pressure of 150/90 mmhg , multiple soft to firm nodular swellings over entire body including face ( straight arrows ) , multiple macules ( arrow heads ) over arms , back , and generalized freckling of the skin ( curved arrows ) known as crowe 's sign [ figure 1 ] . there was no evidence of neck swelling / skeletal deformities / ophthalmic findings and abdominal examination was unremarkable . ultrasonography abdomen revealed a mass lesion from right adrenal gland and ct scan showed a 4.6  3.8 cm mass arising from right adrenal gland with mixed consistency [ figure 2 ] . her intraoperative and postoperative courses were uneventful . during last review 1 month after surgery her blood pressure is now controlled with single drug and repeat urinary metanephrines returned to normal range . our patient is unique in several ways that she never had classical symptoms of pheochromocytoma , unilateral lesion in the setting of familial syndrome and presentation with pain abdomen at a delayed age in sixth decade of life . nf1 is characterized by cutaneous pigmentary abnormalities and neoplastic growth of neural crest - derived cells . other phakomatoses with predilection for endocrinal tumors are tuberous sclerosis , cowden 's syndrome and peutz - jeghers syndrome . multiple endocrine syndromes were divided into two categories ( men1 , men2 ) initially based on the tumoral involvement . however , increasing awareness , genetic and hormonal screening expanded the spectrum to involve six syndromes with multiple subvariants . they are men1 , men2 , vhl disease , nf1 , carney 's complex ( cc ) , and mccune - albright syndrome ( mas ) . they affect endocrine and non - endocrine tissues and most of them have characteristic dermatological findings . hormone overproduction is characteristic of men1 and 2 whereas others present with non - hormonal tumors . our case presented neither with hormone overproduction nor with non - hormonal tumors and had a successful result post - adrenalectomy . pheochromocytoma is seen in about 1% of nf1 patients but the incidence increases to 2050% in cases of nf1 with hypertension . although our patient had clinical features of nf1 and long standing history of hypertension requiring three drugs , the diagnosis was delayed due to the lack of symptoms of the disease . recent reports showed that pheochromocytoma is seen in 7 out of 48 patients with nf1 . clinical presentation remained similar to sporadic pheochromocytoma in six cases except for one case which was asymptomatic similar to our case . previous published reports showed that pheochromocytoma occurs in third to fourth decade but our patient presented in sixth decade of life.[24910 ] nf1-associated pheochromocytoma has germline nf1 mutations that favor the cysteine serine - rich domain and behaves like a sporadic pheochromocytoma except for increased risk of malignancy . persistent hypertension is seen in about 60% of cases of nf1 with pheochromocytoma as seen in our patient . a long - term follow up is required to monitor for recurrence in patients with genetic predisposition . data from observational studies suggest that all patients with nf1 and hypertension should undergo a biochemical testing for pheochromocytoma . to conclude , we report the atypical presentation of pheochromocytoma in an elderly lady with neurofibromatosis . our case highlights the role of screening for pheochromocytoma in all patients of neurofibromatosis with hypertension .
von hippel  lindau ( vhl ) disease includes a wide spectrum of highly vascular tumors like pheochromocytoma , cysts and adenomas of the pancreas and kidney , endolymphatic sac and renal cell carcinoma . 
 molecular analysis of the vhl gene is vital in these patients and their kindred . 
 neuroendocrine pancreatic tumors are rare and are known for their malignant and metastatic potential . 
 we discuss an interesting case of a young lady who had a strong family history of central nervous system and spinal tumors . 
 our patient presented with recently detected severe hypertension . 
 she was found to have elevated urinary normetanephrine levels ; radiological imaging revealed bilateral pheochromocytomas with a pancreatic lesion . on genetic analysis , a variation , c.ivs 2 + 3 a > g , 
 was found in the intronic region following exon 2 of the vhl gene . 
 this variant appears novel . 
 this patient underwent bilateral adrenalectomy ( right total and left cortical sparing ) , distal pancreatectomy and splenectomy . 
 histology was diagnostic for pheochromocytoma with a neuroendocrine tumor of the pancreas . 
 the postoperative period was uneventful . 
 she was advised genetic screening of all her first - degree relatives .