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Melorheostosis with renal arterio-venous malformation: A case report with review of literature.

Lone AR, Ahmad M, Aziz SA, Bhat GM, Bhat JR, Jahan R, Khan SH - Indian J Med Paediatr Oncol (2009)

Bottom Line: The appearance is of "candle greasing" down one side of one or several bones of the body.We describe a case referred to tertiary care center with suspicion of renal cell carcinoma with diffuse bone metastasis.He was reassured about the benign nature of the disease and is asymptomatic.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Oncology, RCC, Sheri Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India.

ABSTRACT
Melorheostosis, also known as Leri's disease and flowing periosteal hyperostosis, is a rare cause of pain and stiffness in a limb. The appearance is of "candle greasing" down one side of one or several bones of the body. We describe a case referred to tertiary care center with suspicion of renal cell carcinoma with diffuse bone metastasis. After reassessment, the patient was diagnosed melorheostosis with renal AV malformation. He was reassured about the benign nature of the disease and is asymptomatic.

No MeSH data available.


Related in: MedlinePlus

(a) Tc-99m DTPA renogram (PA). Tc-99m DTPA renogram (AP); (b) Tc-99m DTPA renogram (AP)
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Figure 0001: (a) Tc-99m DTPA renogram (PA). Tc-99m DTPA renogram (AP); (b) Tc-99m DTPA renogram (AP)

Mentions: A 42-year-old male patient presented in 2001 with urinary symptoms. His physical examination was unremarkable, with normal pulse and blood pressure. Investigation at presentation revealed a normal hemogram and erythrocyte sedimentation rate (ESR). Fasting blood sugar was 90 mg/dL (normal, 90-110 mg/dL), and kidney functions were normal. Ultrasonography revealed grade one benign prostatic hyperplasia with multiple cysts in left kidney. Isotopic Renogram done after intravenous injection of 5 milli Curies of Technetium-99m diethylene triamine penta acetic acid (Tc-99m DTPA) under a large field of view Gamma camera revealed a relative renal function of 45% in left kidney and 55% in the right kidney [Figure 1]. The overall scan impression was that of an enlarged left kidney with mild functional impairment. On reevaluation in 2003, his physical examination was unremarkable. Investigations revealed a normal hemogram, normal results of kidney function tests and normal levels of serum and urinary calcium. Parathormone (PTH) levels were within normal limits. Intravenous pyelogram (IVP) revealed a well-defined cystic lesion in the left kidney near pelvicalyceal system causing splashing of pelvis, obstructed calyces and caliectasis. Contrast-enhanced CAT scan of abdomen (CECT) revealed large multiloculated cystic mass arising from left kidney, impinging on pelvis and causing hydronephrosis [Figure 2]. Fine-needle aspiration was inconclusive. Tc-99m Methylene Diphosphonate (MDP) bone scan revealed abnormal tracer uptake in multiple ribs, femur, pelvis, foot bones, tibia and 9th, 10th and 11th dorsal vertebrae [Figure 3]. Hemogram and serum chemistry were normal. Left radical nephrectomy was done in April 2003. Operative findings revealed a mass involving left kidney, with hydronephrotic changes. Histopathological examination (HPE) revealed arterial malformation of kidney [Figure 4]. Patient was referred to tertiary care center with an impression of renal cell carcinoma with diffuse bone metastasis. He was reevaluated and found to have high serum alkaline phosphatase (Alp) levels. In view of the long history, good performance status and histopathological examination, skeletal survey was done, which revealed hyperostosis of long bones, and the bone of foot resembling wax dripping on one side [Figure 5]. A final diagnosis of melorheostosis was made and the patient reassured. He is on our follow-up for the last 5 years and is asymptomatic.


Melorheostosis with renal arterio-venous malformation: A case report with review of literature.

Lone AR, Ahmad M, Aziz SA, Bhat GM, Bhat JR, Jahan R, Khan SH - Indian J Med Paediatr Oncol (2009)

(a) Tc-99m DTPA renogram (PA). Tc-99m DTPA renogram (AP); (b) Tc-99m DTPA renogram (AP)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2902215&req=5

Figure 0001: (a) Tc-99m DTPA renogram (PA). Tc-99m DTPA renogram (AP); (b) Tc-99m DTPA renogram (AP)
Mentions: A 42-year-old male patient presented in 2001 with urinary symptoms. His physical examination was unremarkable, with normal pulse and blood pressure. Investigation at presentation revealed a normal hemogram and erythrocyte sedimentation rate (ESR). Fasting blood sugar was 90 mg/dL (normal, 90-110 mg/dL), and kidney functions were normal. Ultrasonography revealed grade one benign prostatic hyperplasia with multiple cysts in left kidney. Isotopic Renogram done after intravenous injection of 5 milli Curies of Technetium-99m diethylene triamine penta acetic acid (Tc-99m DTPA) under a large field of view Gamma camera revealed a relative renal function of 45% in left kidney and 55% in the right kidney [Figure 1]. The overall scan impression was that of an enlarged left kidney with mild functional impairment. On reevaluation in 2003, his physical examination was unremarkable. Investigations revealed a normal hemogram, normal results of kidney function tests and normal levels of serum and urinary calcium. Parathormone (PTH) levels were within normal limits. Intravenous pyelogram (IVP) revealed a well-defined cystic lesion in the left kidney near pelvicalyceal system causing splashing of pelvis, obstructed calyces and caliectasis. Contrast-enhanced CAT scan of abdomen (CECT) revealed large multiloculated cystic mass arising from left kidney, impinging on pelvis and causing hydronephrosis [Figure 2]. Fine-needle aspiration was inconclusive. Tc-99m Methylene Diphosphonate (MDP) bone scan revealed abnormal tracer uptake in multiple ribs, femur, pelvis, foot bones, tibia and 9th, 10th and 11th dorsal vertebrae [Figure 3]. Hemogram and serum chemistry were normal. Left radical nephrectomy was done in April 2003. Operative findings revealed a mass involving left kidney, with hydronephrotic changes. Histopathological examination (HPE) revealed arterial malformation of kidney [Figure 4]. Patient was referred to tertiary care center with an impression of renal cell carcinoma with diffuse bone metastasis. He was reevaluated and found to have high serum alkaline phosphatase (Alp) levels. In view of the long history, good performance status and histopathological examination, skeletal survey was done, which revealed hyperostosis of long bones, and the bone of foot resembling wax dripping on one side [Figure 5]. A final diagnosis of melorheostosis was made and the patient reassured. He is on our follow-up for the last 5 years and is asymptomatic.

Bottom Line: The appearance is of "candle greasing" down one side of one or several bones of the body.We describe a case referred to tertiary care center with suspicion of renal cell carcinoma with diffuse bone metastasis.He was reassured about the benign nature of the disease and is asymptomatic.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Oncology, RCC, Sheri Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India.

ABSTRACT
Melorheostosis, also known as Leri's disease and flowing periosteal hyperostosis, is a rare cause of pain and stiffness in a limb. The appearance is of "candle greasing" down one side of one or several bones of the body. We describe a case referred to tertiary care center with suspicion of renal cell carcinoma with diffuse bone metastasis. After reassessment, the patient was diagnosed melorheostosis with renal AV malformation. He was reassured about the benign nature of the disease and is asymptomatic.

No MeSH data available.


Related in: MedlinePlus