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Indian Journal of Surgery
Medknow Publications on behalf of Association of Surgeons of India
ISSN: 0972-2068
Vol. 67, Num. 2, 2005, pp. 106-108

Indian Journal of Surgery, Vol. 67, No. 2, March-April, 2005, pp. 106-108

Case Report

Melorheostosis: Follow-up of a case Compounded with carcinoma prostate

District Hospital, Durg, Consultant, Surgeon, Naik Nursing Home, Arya Nagar, Durg (C.G.) - 491 001
Correspondence Address:District Hospital, Durg, Naik Nursing Home, Arya Nagar, Durg (C.G.) - 491001 Email: mantosh2@rediffmail.com

Code Number: is05027

Abstract

A case of melorheostosis, a relatively uncommon congenital condensing bone disease, developed adenocarcinoma of prostate - a relatively slow-growing tumour usually amenable to treatment with good prognosis. The malignancy in this case disseminated unusually fast spelling out the unfavourable prognosis. This patient also had a fracture neck of the femur which unexpected healed very early. We believe that these observations support the notion of the congenital local hypervascularity theory in the aetiology of the disease.

Keywords: Melorheostosis, TURP, secondaries, prostatectomy, orchiectomy

Introduction

Melorheostosis, a rare congenital condensing bone disease, characterized by a flowing type of hyperostosis resembling "candle flow streaming" in the long bones, may be monomelic and polyostotic. It is easily recognizable radiographically by irregular cortical thickening, periosteal proliferation and sclerosis that blends with irregular cortex involving one side of bone. The location is usually diaphyseal. The aetiology is unknown and the onset is usually insidious. [1],[2],[3] Ordinarily, the prognosis of the disease is good.[4]

The present case was initially reported with reference to a fracture neck femur with early healing of fracture, despite inadequate immobilization, supporting the hypervascularity theory originally proposed by Lorimier and Moore.[1],[5] We would like to clarify that this particular patient was reported earlier (1979) but this communication deals with another disorder that provides support for hypervascularity in the causation of the disease.

Case Report

A 58-year-old male was a known case of monomelic, polyostotic type of melorheostosis[1] and was followed up diligently. In April 2000 the patient sought medical attention for complaints of increased frequency and difficulty in passing urine with distended bladder. He was provisionally diagnosed as a case of benign hyperplasia of prostate.

Digital rectal examination revealed prostatomegaly with flat anterior rectal wall mucosa, firm to hard in consistency at places, and the median sulcus was obliterated. A Foley's catheter was placed for the continuous drainage of urine and further investigations and procedures were carried out as listed in [Table - 1].

Discussion

This case is singularly interesting because firstly, it provided an opportunity for a long follow-up. Secondly, the natural process was complicated by two unusual events with unlikely outcomes, namely a fracture femoral neck that healed and rapid metastatic spread from a prostatic malignancy with low metastatic potential. More than 90% of prostatic metastases are osteoblastic.[4],[6] Secondaries may be so extensive as to produce diffuse chalkiness of all bones. Osteolytic lesions are rare and may be solitary.[7] It is believed that the metastatic process follows a network of valveless veins in the pelvis including the veni vasorum of the extremities and their tributaries. These veins go directly posterior to the sacral region, and also to the pelvis carrying tumour metastases with them.[6] The earlier area of bone involvement is on the inner aspect of the acetabulum, and it spreads with similar propensity in all directions from this site. [Figure - 1], [Figure - 2], [Figure - 3]

Carcinoma of the prostate is usually spheroidal-celled, with a varying degree of tubule formation and slow growth. Curiously enough, in the present case, it rapidly metastasised locally through lymphatic and venous channels. We feel it is reasonable to suggest that the hypervascularity of the pelvic bone due to melorheostosis explains the early and widespread metastases of the disease. Along with the earlier report of primary healing in a femoral neck fracture this finding supports the concept of hypervascularity as an aetiological factor in the development of melorheostosis.

No malignant degeneration of the affected segment of bone with melorheostosis has been reported so far.2 However, more recently two cases of osteosarcoma in melorheostotic femur have been reported.[8],[9] Radiographs of this patient illustrate an unusual radiological combination of melorheostotic pelvic bone with secondary deposits from prostatic carcinoma in the pelvic bones.

Wahi et al[10] have discussed at length the theories, viz. infective theory ( l0 eri), developmental disorder (Zimmer and Junghagen), ischaemic theory (Putty) telangiectatic theory (Lorrimier and Moore), etc. Among these theories the one suggesting "developmental disorder" seems to be the most acceptable. Campbell et al[2] have also pointed out the various aetiological theories for this disorder. They clearly point out the non-hereditary and congenital origin of the disease. The authors find malformation of the limb bud to be the most plausible aetiology of the disorder, and do not preclude involvement of the metameric mesoderm, some cells of which may have migrated to the limb bud. In view of the recorded history of this case, which involves differential course in two disorders viz. traumatic and malignant, over a period of 25 years, all that can be reasonably said is that there seems to have been maldevelopment of the mesoderm involving unilateral localised hypervascularity - mechanisms that have been suggested by Lorimier and Moore.[5]

With the present state of knowledge all that can be said with reasonable certainty is that melorheostosis is congenital in origin and involves unilateral maldevelopment of the mesoderm with an element of hyervascularity. Previous authors have suggested hypervascularity as a causative factor in the development of melorheostosis.[5] The present case has offered two events to support this suggestion, first a subcapital fracture of neck of femur on the affected side that healed rapidly and second, early dissemination of an adenocarcinoma of the prostate, which is generally slow-growing. Melorheostosis is a benign disorder and compatible with normal life expectancy, as the disease has been followed from childhood to the termination of life at the age of 58. The early termination may be ascribed to carcinoma of prostate. This case further highlights the need for a close follow-up of patients with melorheostosis.

References

1.Naik RS, Tiwari NP. Melorheostosis associated with fracture neck femur. Indian J Surg 1979;41:535-9.  Back to cited text no. 1    
2.Campbell CJ, Papademetriou T, Bonfiglio M. Melorheostosis. A report of the clinical, Roentgenographic, and pathological findings in fourteen cases. J Bone Joint Surg Am 1968;50A:1281-304.  Back to cited text no. 2    
3.Greenspan A, Azouz EM. Bone dysplasia series. Melorheostosis: Review and update. Can Assoc Radial J 1999;50:324-30.  Back to cited text no. 3  [PUBMED]  
4.Green AE Jr, Ellswood WH, Collins JR. Melorheostosis and Osteopoikilosis, with a review of the literature. Amer J Roentgenol 1962;87:1096-111.  Back to cited text no. 4  [PUBMED]  
5.Moore JJ, de Lorimier AA. Melorheostosis Leri - Review of literature and report of a case. Amer J Roentgenol 1933;29:161-71.  Back to cited text no. 5    
6.Meschan Isadore. Roentgen Signs in Clinical Practice, Vol. 1, 2nd Ed. London: W. B. Saunders Company; 1966. p. 361-26.  Back to cited text no. 6    
7.Russell RCG, williams NS, Bulstrode CJK. Bailey and Love's short practice of surgery, 23rd Ed. London: Arnold Publishers; 2000. p. 1249-54.  Back to cited text no. 7    
8.Bostman OM, Holmstrom T, Riska EB. Osteosarcoma arising in a melorheostotic femur: A case report. J Bone Joint Surg Am 1987;69-A:1232-7.  Back to cited text no. 8    
9.Murphy M, Cavanagh M, Hurson B, Kearns S, O'connell D. Occurence of osteosarcoma in a melorheostotic femur, IMJ 2003;96:1-4.  Back to cited text no. 9    
10.Wahi PN, Mehrotra MP, Haldar PK, Lehri B, Kumar P. Melorheostosis, J Assn Phys India 1963;11:301-8.  Back to cited text no. 10    

Copyright 2005 - Indian Journal of Surgery


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