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East and Central African Journal of Surgery
Association of Surgeons of East Africa and College of Surgeons of East Central and Southern Africa
ISSN: 1024-297X EISSN: 2073-9990
Vol. 13, Num. 1, 2008, pp. 66-72
Contents

East and Central African Journal of Surgery, Vol. 13, No. 1, March-April 2008, pp. 66-72

Childhood Bone and Soft Tissue Tumours: A review of 43 Treated at two University Teaching Hospitals in Rwanda (CHUB and CHUK) in RWANDA

M. Makanga, J.P. Majyambere, I. Kakande 

Department of Surgery, Butare Teaching Hospital. National University of Rwanda.
Correspondence to:Dr Martine Makanga Email:mmaka1@yahoo.fr

*Presented at The Association of Surgeons of East Africa Conference at Mombasa December 2007

Code Number: js08011

Back ground: Cancer represents one of the major causes of death in the world estimated at 1 out of 10 deaths. Globally, 160,000 children are diagnosed to have cancer each year. In the developing countries, one child out of two with cancer will die because of this disease mostly because they present with advanced disease or due to limited resources for proper management1,2. This study was aimed at determining the prevalence, the histological types and the management outcome of childhood bone and soft tissue tumours in Rwanda
 Methods: A retrospective descriptive study of 43 histologically confirmed cases of bone and soft tissue tumours in children was done in the Surgery Departments of University Teaching Hospitals (CHUB and CHUK). The period of study was 6 years from January 2001 to December 2006. Only children aged 16 years or below were included in the study. Data was obtained from patient’s clinical files, pathology register and theatre registers. Information obtained was recorded using a special questionnaire; Data obtained was analyzed using Epidata 2.1b, SPSS 11.5 computer programs. Statistic test Pearson Chi-carré (P) was considered significant if P value was less than or equal to 0.05.
 Results: During 6 years period, 43 children aged 2 to 16 years were seen having bone and soft tissue tumours. Males accounted for 28 (65.1%) of the cases. The 10 to 16 year age group was predominantly affected, accounting for 72% (n=31) of all cases.. Pain and swelling were the main clinical signs in 100%of cases. Tumours were benign in 17 (40%) and malignant in 26 (60%) of cases. History of local trauma and pain was associated with malignant bone tumours (P=0.003 and P=0.000), respectively (n=23). Delay time between the onset of symptoms and consultation to hospitals was associated with death for malignant tumours (P=0.049). The bones were affected in 76.7% (n=33),  being malignant in 23 (69.7%)of them. The hospital mortality rate was 18% for malignant tumours. Pulmonary and bone spread of the disease were observed in all fatal cases.
Conclusion:  Limitation of diagnostic and therapeutic means and lack of an oncology department that would provide chemotherapy and radiotherapy) in our University teaching hospitals (CHUB and CHUK) made the management of malignant tumours and the follow up after discharge inadequate, unsuitable and  difficult. 

Introduction

Although, Cancer is rare in children aged less than 15 years1, it represents a main concern for parents, families, and health professionals to care for children affected with cancer. The problems confronting Paediatric oncology in Africa are many. They are linked to:

  • Lack of common strategy and policies concerning detection, prevention, and treatment of cancer patients
  • Insufficient specialized human resources,
  • Absence of referral cancer centers,
  • Unavailability of antimitotic drugs and radiotherapy,
  • Lack of financial resources
  • Patients consulting at late stage of cancer.

These lead to low survival and cure rates: The patients’ survival at 5 years is ≤ 50% in developing world whereas cure rates in developed countries represent 75-80 %1. In Rwanda there is no possibility of effective, efficient management of cancer after diagnosis is confirmed for lack of an established oncology Department. A review of literature shows that no specific study on cancer in children has bee reported from Rwanda. It was with such background that a study was undertaken on childhood cancers in Butare university Teaching Hospital.

Methods

A retrospective descriptive study of 43 histologically confirmed cases of bone and soft tissue tumours in children was done in the Surgery Departments of University Teaching Hospitals (CHUB and CHUK). The period of study was 6 years from January 2001 to December 2006. Only children aged 16 years or below were included in the study. Data was obtained from patient’s clinical files, pathology register and theatre registers. Information obtained was recorded using a special questionnaire; Data obtained was analyzed using Epidata 2.1b, SPSS 11.5 computer programs. Statistic test Pearson Chi-carré (P) was considered significant if P value was less than or equal to 0.05.

Results

During the 6 years under review, bone and soft tissue tumours accounted for  2% in CHUB and 1.8% in CHUK  of  paediatric surgery admissions. A total of 43 children we admitted to the two University teaching hospitals at Butare (CHUB) and Kigali (CHUK). The majority of 58.1% (n=25) were admitted in CHUB (Table 1). Table 2 shows the age distribution. The 10 to 16 age group years contributed 31 (72%0 0f all cases. Males were 28). The male to female sex ratio was 1.9: 1. Twenty four patients came from Southern province in which CHUB is located.

The time interval between the onset of symptoms and consultation to the  university teaching hospitals ranged between 2 and 36 months.

Table2: Age group distribution

Age in Years

Number

%

<5

3

7,0

5-10

9

21,0

10-16

31

72,0

Total

43

100

Table3: Patients clinical signs

Clinical Feature

Number

%

Swelling

43

100

Pain

29

67.4

Local injury

6

14

Bad general condition

3

7.0

Fever

1

2.3

Pathological fracture

1

2.3

Dyspnea)

1

2.3

Skin ulceration

1

2.3

Lymph nodes

1

2.3

Swelling

43

100.0

Table 4. Distribution of Affected Long Bones

Bone

 

Number of Times

 

%

Femur

12

43

Tibia

11

39

Humerus

2

7

Ulna

2

7

Radius

1

4

Total

28

100

Table 5. Distribution of Malignant Tumours

Histological type

 

Frequency

 

%

Malignant histiocytofibroma

2

7.7

Chondrosarcoma

6

23

Fibro sarcoma

1

3.8

Osteosarcoma

14

54

Rhabdomyosarcoma

2

7.7

Ewing’ s Sarcoma

1

3.8

Total

26

100

Table 6. Distribution of benign tumours

Histological type

Frequency

 

%

Chondroma

2

11.8

Osteochondroma

6

35.2

Soft tissue fibroma

3

17.7

Lipoma

4

23.5

Osteoma

2

11.8

Total

17

100

A significant number (30.2%) consulted between 6 to 12 months. The  average delay was 12 months. Clinical signs were mostly swelling in 100% (n=43) and pain in 67.4% (n=29). History of local trauma was elicited from 6 patients having malignant bone tumours (26%) with P=0.003. Bone tumours were 33 (76;7%) and soft tissue tumours were 10 (23). The long bones were affected in 28 (65.1% ) of the children with the lower limbs bones (Femur and tibia) being more involved than the upper limb (Table 4). Soft tissue tumours were mostly located on the shoulder (n=3), buttocks, arm and thigh (n = 2 each) and forearm (1 case). All patients had tumour biopsy. The bone X-rays were done in 34 (79%), chest X-ray in 26 (69.7%) and ultrasonography in 9 (20.9%).

Table 7. Distribution of Bone Tumours

Histological type

     Benign

 

Malignant

%

Malignant histiocytofibroma

 

2

6.1

Chondroma

2

 

6.1

Osteochondroma

6

 

18.2

Chondrosarcoma

 

6

18.2

Osteoma

2

 

6.1

Osteosarcoma

 

14

42.3

Ewing’sarcoma

 

1

3.0

Total

10 (30.3%)

23 (69.7%)

100.0

Of the 32 patients whose HIV status was known, only 2 (4.6) were positive. Five (19.2%) of the s6 patients who had chest xrays already had pulmonary metastases on their admission. Malignant tumours were diagnosed in 26 (60%) (Table 5). Of these, osteosarcomas were 14 (54%). Osteochondromas were the commonest benign tumours (Table 6)

In our study, most of bone tumours were malignant 69.7% (n=23).Osteosarcoma was the most frequent bone tumour 42.3% (n=14). Among malignant bone tumours, femur was the most affected bone in 50% (n=10), followed by tibia in 40% (n=8) and humerus in 10% (n=10). There was a significant relation between pain and bone malignant tumours. All patients having bone malignant tumours had pain (P=0.000). Among patients having bone and soft tissue tumours, the age group from 10 to16 years was the most affected 72% (n=31). Osteosarcoma was the most common tumour in this age group accounting for 12 {38.7%) of cases.

Twenty (46.5%) of the patients had surgical treatment that included tumour excision for benign tumours; limb amputation in 13 (30.2%) or disarticulation in 7 (16.3%) of the patients with malignant tumours. Three patients (7%) in bad general conditions with metastatic malignant tumours had a palliative treatment (analgesics, oxygen). None of the patients received chemotherapy or radiotherapy.

On discharge 30 (69.7%) of the patients had clinical improvement. There was a significant relation between the histological type of the tumour and death (P=0.042). Six patients all with malignancies died that is 14% of all cases or 23% of all malignancies.  Four deaths had Osteosarcoma, one had Ewing’s tumour and one had a chondrosarcoma. The presence of distance disease extension was the determinant factor to patient death (P=0.001). All the 6 deceased patients had pulmonary metastases, 3 of them had also bone extensions.

The length stay patients in hospital varied from 2 to 26 days. The average was 10 days for benign tumours and 16 days for malignant tumours. There was a significant relation between the long stay in hospital and the malignancy of the tumour (P=0,031).Patients follow up was difficult. only 11 patients (35.5%); 8 patients (25,8%) operated for benign tumours, 3 patients (9.7%) with malignant tumours, came back for review after discharge. Two patients had a referral for chemotherapy and radiotherapy abroad. The majority of patients were lost to follow up. It was therefore difficult to know if they were still alive or had died.

Table 8. Age Group Distribution and Histological Types

Histological Types

Age Groups

Total

2-5 Years

5-10 Years

10-16 Years

2-16 Years

Chondroma

0

0

2

2

Osteochondroma

0

1

5

6

Chondrosarcoma

0

0

6 (19.3%)

6

Fibroma

0

3

0

3

Fibrosarcoma

0

1

0

1

Malignant Histiocytofibroma

0

0

2

2

Lipoma

0

2

2

4

Osteoma

0

0

2

2

Osteosarcoma

0

2

12 (38.7%)

14

Rhabdomyosarcoma

2

0

0

2

Ewing’sarcoma

1

0

0

1

Total

3   (7%)

9 (21%)

31 (72%)

43

Table 9. Patient Treatment Distribution

Treatment

Frequency

%

Tumour excision

20

46.5

Amputation

13

30.2

Disarticulation

7

16.3

Palliative care

3

7

Chimiotherapy

0

0

Radiotherapy

0

0

Total

43

100

Discussion

According to the literature review the age group from 10 to 16 years s the most affected3,4,5,6. The males are predominantly affected by bone and soft tissue tumours7,8,9,10. There is no clear explanation for the sex differences. Most of our patients had a long delay prior to coming for consultation. This is in contrast to reports from the more developed countries like USA11, Australia7, Serbia5 and Poland9,where patients consult earlier. The long delay among our patients may be explained by the low socio-economical of the affected families. For lack of funds for hospital fee and transport, many of our patients commonly choose to first consult traditional healers.

The clinical features of malignant tumours in our study were similar to findings reported by Cleeman11 in USA, Bacci6 in Italy, Tanz7 in Australia, and Brown12 in Nigeria, on the bone predominant localisation of malignant tumours. The limited investigative capacities in our institutions characterized by lack of appropriate imaging facilities insufficient reagents for laboratory tests compounded by shortage of trained manpower are major contributory factors to delayed diagnosis and treatment. Our poor investigations are in contrast to what exists in other centres  where CT scanner is used to guide biopsy and tom densitometry, bone scintigraphy and biochemical tests are performed13,14,15,16,17. The histological types found in our series and their predominance are similar to what other studies by Settakorn18 in Thailand, Cleeman11 in USA, Bacci6 in Italy, Brown12 in Nigeria, and Blakwell19 in Australia reported.

Management of malignant tumours is poor and almost confined to surgical treatment whenever possible and palliative care for the advanced disease. Absence of an established oncology department in our two top referral centres is a major obstacle to proper management of our cancer patients.  In other centres adjuvant chemotherapy and radiotherapy significantly affect the outcome of cancer treatment12,11,7,6.

There an urgent need to improve the diagnostic facilities, to train more pathologists, oncologist and specialized personnel in palliative care. An Oncology Department should be established in Rwanda and a multidisciplinary approach to management of cancer patients (involving surgeons, oncologists, radiotherapists, palliative care specialists and nurses, counsellors and psychologists etc)  should be promoted.  Lastly, a National Cancer Registry should be set up.. All these measures will go a long way in improving the outcome of management of cancer patients in Rwanda.

References

  1. Centre International De Recherche Sur Le  Cancer.Publications scientifiques No144 et www-dep.iarc.fr/accis.htm ; Institut National du cancer.2004International Union against Cancer. (Consulté le2/2/2006).
  2. http://seer.cancer.gov/csr/1975_2000/results_merged/sect_28_childhood_cancer_iccc.pdf (Consulté le 18/1/2006).
  3. James G. Gurney, Andrine R. Swensen, Marc Bulterys: Malignant bone and soft tissue tumors in childhood. National cancer institute. SEER Pediatric Monograph.2006: 99-110.
  4. Nomikos GC, Murphey MD, Kransdorf MJ, Bancroft LW, Peterson JJ. Primary bone tumors of the lower extremities.Radiol Clin North Am. 2002 Sep; 40(5):971-90. Review Department of Radiology, New York University Medical Center, 550 First Avenue, New York NY 10016, USA.
  5. Stevanovic V, Vukasinovic Z, Spasovski D. Bone tumors-analysis of epidemiological parameters and their relation to clinical characteristics Srp Arh Celok Lek. 2006 Jul-Aug; 134(7-8):310-4. Serbian.
  6. Bacci G, Longhi A, Versari M. Prognostic factors for osteosarcoma of the extremity treated with neoadjuvant chemotherapy: 15-year experience in 789 patients treated at a single institution. Department of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy. Cancer. 2006 Mar 1; 106(5):1154-6.
  7. Tan JZ, Schlicht SM, Powell GJ, Thomas D, et al. Multidisciplinary approach to diagnosis and management of osteosarcoma - a review of the St Vincent's Hospital experience.Int Semin Surg Oncol. 2006 Nov 3; 3:38. Melbourne, Australia. (Abstract)
  8. Faisham WI, Zulmi W, Halim AS et al. Osteosarcoma: the outcome of limb salvage       surgery Musculoskeletal Oncology Unit, School of Medical Science, Universiti Sains Malaysia, Kubang Kerian, Kelantan : Med J Malaysia. 2004 Dec;59 Suppl F:24-34.
  9. Szumera M, Czauderna P, Popadiuk S, Renke J, Pulmonary metastases in children with solid tumours - own experiences. : Med Wieku Rozwoj. 2006; X (3 pt 1):665-675 Klinika Pediatrii, Gastroenterologii i Onkologii Dzieciecej, Akademia Medyczna, ul. Nowe Ogrody 1-6, 80-803 Gdansk, Poland.
  10. Iagaru A, Quoins A, McDougall IR, Gambhir SS. F-18 FDG PET/CT evaluation of osseous and soft tissue sarcomas Division of Nuclear Medicine, Department of Radiology, Stanford Hospital and Clinics, Stanford, California, USA. Clin Nucl Med. 2006 Dec; 31(12):754-60
  11. Cleeman E, Auerbach JD,    Springfield DS. Musculosqueletal tumors: a review of 194 cases.J Shoulder Elbow Surg. 2005 Sep-Oct; 14(5):460-5. Manhattan Orthopaedic & Sports Medicine Group, Mount Sinai Medical Center, New York, NY 10029, USA.
  12. Brown BJ, Oluwasola AO. Childhood bone and soft tissue tumors in Ibadan, Nigeria : 1984-2003. Department of Paediatrics, Faculty of Clinical Sciences, College of Medicine, University of Ibadan/University College Hospital, Ibadan, Nigeria.Ann Trop Paediatr. 2006;26(4):349-55.
  13. Hans P. Houegger.Tumeurs et sarcomes des parties molles : Ligne suisse contre le cancer. Disponible sur : http: // www. Prevention.ch/ tumeurs et sarcomes. Htm. (consulté le 5. 02. 2007)
  14. Mirra JM, Picci P, Gold RH. Bone tumours: clinical radiologic and pathologic correlation. Philadelphia: Lea and Febiger, 1989.
  15. Petterson H, Hamlin DJ, Mancuso A, Scott KN. Magnetic resonance imaging in musculoskeletal system. Acta Radiol Diagn 1985; 26:225-234.
  16. 16.  
  17. Arnette Blackwell. Standard-Option-Recommandations: Sarcomes des tissus moux et ostéosarcomes. Vol 1. Paris, 1995.
  18. http://www.med.univ-rennes1.fr/cerf/edicerf/OSTEO-ARTICULAIRE/07OSTEO.html.       ( Consulté le29/11/2005)
  19. Settakorn J, Lekawanvijit S, Arpornchayanon O, et al. Spectrum of bone tumors in Chiang Mai University Hospital, Thailand according to WHO classification 2002: A study of 1,001 cases.
  20. Blackwell JB, Threlfall TJ, McCaul KA. Primary malignant bone tumours in Western Australia, 1972-1996. : Pathology. 2005 Aug; 37 (4):278-83.(Full text)

© 2008 East and Central African Journal of Surgery


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