CHONDROMYXOID FIBROMA

ORTHO-PATHO MEET

1. ORTHO-PATHO MEET PRESENTER: DR. MURUGESH M KURANI Dept. of Orthopaedics, J N Medical College, BELAGAVI .

2. CLINICAL HISTORY Name: xyz Age: 53 years. Sex: Female. Occupation: Housewife.

3. CHIEF COMPLAINTS C/O swelling in the right ring finger since 3 months C/O pain in the swelling since 3 months

4. HISTORY OF PRESENTING ILLNESS Patient was apparently alright 3 months back, later she noticed a small swelling in the right ring finger which was associated with mild intermittent pain. Swelling gradually progressed to attain present size of around 1.5x1 cm . Since last 15days pain increased in severity and is continuous in nature. Pain is more during night time.

5. Pain increases on movements and relieves upon rest No h/o trauma/ fall No h/o fever/ weight loss/ decreased appetite No h/o intake of any medication

6. PAST HISTORY: No history of similar complaints in the past. No h/o -ASTHAMA/TB / brucellosis No h/o DM/HTN FAMILY HISTORY: No h/o similar complaints in the family

7. PERSONAL HISTORY Diet : vegeterian Appetite : Adequate Sleep : decreased because of pain Bowel & Bladder : Normal and regular No h/o of smoking /tobacco/alcohol intake

8. GENERAL PHYSICAL EXAMINATION An elderly female patient , moderately built & nourished, conscious and co-operative & well oriented to time, place & person. VITALS: PR: 82/min BP: 134/86 mm of Hg in supine RR: 19 /min Temp- afebrile 98*F

9. Pallor: absent Icterus : absent Cyanosis: absent Clubbing: absent Lymphadenopathy : absent Edema: absent

10. SYSTEMIC EXAMINATION CVS: S1 S2 + ,no murmur RS : B/L Normal vascular breath sounds present B/L Air entry equal. PA : Soft, Non tender, Bowel sounds present, no organomegaly . CNS: No focal neurological deficit.

11. LOCAL EXAMINATION INSPECTION: Localised ovoid swelling of around 1.5X1 cm over middle phalynx of right ring finger on volar aspect. (parallel to long axis of bone) Skin over the swelling is stretched. No scar/ sinus/ redness. No visible pulsations/ dilated veins.

12. PALPATION local rise of temperature. Tenderness present over swelling. Size: 1.5x1 cm, hard, fixed to underlying bone . No distal neuro -vascular deficit.

13. MOVEMENTS: Flexion at PIP joint terminally restricted.

14. INVESTIGATIONS: Hb : 11.4g% WBC : 6800 cells/ cumm , N62, L 30, E 06, M 02 PLATELET COUNT : 3.05L/ cumm RBS : 116 mg% UREA : 29mg% CREATININE: 1.1 mg% HIV, HBsAg , HCV : NON REACTIVE

15. X RAY OF RIGHT HAND Sharply marginated , lobulated,eccentrically located lucent defect in metaphysio-epiphysial region of middle phalynx of ring finger.

16. MRI OF RIGHT HAND IMPRESSION: Well defined signal abnormality along palmar aspect of ring finger in the region of middle phalynx s/o GCT of tendon sheath d/d ganglion cyst.

17. DIFFERENTIAL DIAGNOSIS GANGLION CYST GCT OF TENDON SHEATH ENCHONDROMA PERIOSTEAL CHONDROMA SIMPLE BONE CYST ANEURYSMAL BONE CYST CHONDROBLASTOMA CHONDROMYXOID FIBROMA CHONDROSARCOMA

18. GCT TENDON SHEATH A giant cell tumor of tendon sheath consists of multinucleated giant cells, inflammatory cells, histiocytes , and fibroblasts. It develops in or near synovial joints, bursae , and tendon sheaths and may represent a reactive inflammatory process or a benign neoplasm. AGE- It most frequently develops in people between 30 and 50 years of age. SITE- It commonly appears in the hand and less commonly in the foot, ankle, and knee. Physical examination reveals a firm, small, lobulated mass fixed to the underlying tissues or tendon sheaths . Occasionally it can erode bone. This tumor may grow slowly and recur following surgical excision.

19. GANGLION CYST Ganglion cysts are unilocular or multilocular collections of translucent fluid or gelatinous myxoid tissue surrounded by fibrous tissue. AGE- They can occur in patients of any age and are located in a superficial location adjacent to synovial joints or tendon sheaths. SITE - They are commonly found near the wrist, hand, and knee. Occasionally those that develop near the knee grow to a large size and dissect through the surrounding soft tissues. Enlargement of the limb or swelling caused by these unusual ganglia may suggest the presence of a neoplasm. Although aspiration can remove the fluid from a ganglion cyst, surgical resection is currently the most predictable method of eradicating symptomatic lesions.

20. ENCHONDROMA An enchondroma is a benign hyaline cartilage lesion located in the medullary cavity of otherwise normal bones. It frequently occurs in the bones of the hands and feet but may appear in any bone including the femur, tibia, and humerus . It is generally considered an asymptomatic, indolent lesion. Plain radiographs reveal a central, well-circumscribed lucent region that may be mineralized . Enchondromas resemble bone infarcts. Further imaging studies are not necessary, but enchondromas normally show increased activity on a bone scan.

21. During skeletal growth the lesions may slowly enlarge. Following completion of normal growth, they cease to enlarge and the cartilage component calcifies to give a stippled radiographic appearance. In extremely rare cases, a chondrosarcoma can develop from an enchondroma . Because enchondromas are usually asymptomatic and do not enlarge after skeletal maturity, a lesion that causes pain or enlarges in an adult strongly suggests the possibility of malignant transformation. Enchondromas generally do not require surgical treatment.

22. PERIOSTEAL CHONDROMA A periosteal chondroma is a rare, subperiosteal lesion consisting of hyaline cartilage. It forms between the cortical bone and overlying periosteum , often creating an indentation in the bone surface and a smooth bulge of periosteum -covered cartilage that projects into the soft tissues. AGE- Most patients are young or middle-aged adults. Presumably periosteal chondromas develop from proliferation of cartilage-forming periosteal cells. SITE- They occur most frequently in the proximal humeral metaphysis , phalanges, metacarpals, and metatarsals.

23. They usually present as a solitary, painful mass or as an incidental radiographic finding. Radiographs show a scalloped depression in the bone cortex and may show the faint image of a soft tissue mass containing speckled regions of calcification. Periosteal chondromas can slowly enlarge, but they have not been shown to be aggressive. For symptomatic or enlarging lesions, surgical resection provides definitive local control.

24. SIMPLE BONE CYST Simple, or unicameral, bone cysts consist of fluid-filled cavities within bone lined by a thin layer of fibrous tissue. Age- They occur most commonly in children less than 15 years of age. Site- Approximately 50% occur in the proximal humeral metaphysis . Other common sites include the proximal femur and iliac wing. They may cause slight expansion of bone and thinning of the cortex. As a result patients often present with a pathologic fracture through the cyst.

25. Radiographically , simple bone cysts are centrally located, lucent lesions of the metaphysis . As new bone in skeletally immature patients grows away from the cyst, the lesion may eventually reside in the diaphysis . The current recommended treatment of simple cysts includes observation with restriction of activity and steroid injections. Intralesional curettage and bone grafting is generally reserved for large cysts at high risk for fracture in the proximal femur.

26. ANEURYSMAL BONE CYST(ABC) Aneurysmal bone cysts (ABCs) consist of blood-filled cavities lined by fibrous septae that include giant cells and areas of osteoid but no true endothelial cells. AGE- Approximately 85% of patients with ABCs present before age 20 . The most common symptoms are pain, swelling, and tenderness on palpation of the involved bone. SITE- ABCs commonly involve the metaphysis of long bones, posterior elements of the vertebrae, pelvis, or scapula, but they can occur throughout the skeleton. ABCs can grow rapidly and frequently cause pathologic fractures. When located in the spine , they can cause neurologic compromise.

27. Plain radiographs show a lytic lesion causing marked expansion of the involved bone and occasional periosteal new bone formation. An MRI scan often reveals fluid-fluid levels. They may stop expanding and begin to ossify after reaching a certain size or they may regress spontaneously. Standard treatment is a confirmatory biopsy followed by intralesional curettage and bone grafting.

28. CHONDROBLASTOMA A chondroblastoma is a benign cartilage tumor consisting of regions or “islands” of densely packed polyhedral cells called chondroblasts admixed with fibrous tissue and chondrocytes forming a cartilage matrix. Site- epiphysis of long bones in patients with open physes . They occur most commonly in the proximal humerus , distal femur, proximal tibia, and proximal femur.

29. Radiographs typically show an eccentric, epiphyseal lucency with punctate calcifications. A sclerotic rim surrounds the lucent area. The lesions rarely involve more than half of the epiphysis and only occasionally extend into the metaphysis . Intralesional curettage and bone grafting is indicated for a chondroblastoma .

30. TREATMENT Excision and curettage done under regional block.

31. HISTOPATHOLOGY REPORT GROSS EXAMINATION: Single, grey white, firm tissue, measuring 1.2x0.8x0.7cm. Cut section shows grey white appearance. MICROSCOPIC EXAMINATION: Mass lined by lobules of chondrocytes surrounded by fibroblasts along with central areas of ossification. No evidence of malignancy in the sections studied. IMPRESSION: CHONDROMYXOID FIBROMA- MIDDLE PHALYNX OF RIGHT RING FINGER.

32. CHONDROMYXOID FIBROMA Rare, slow growing, benign cartilagenous tumor <1%, malignant degeneration is rare. Charecterised by GRM1 gene fusion or promoter swapping. Associated with a translocation at t(1;5) Described in 1943 by Jaffe and Lichtenstein , who differentiated the histologic findings from chondrosarcoma , before 1943 it is considered a giant cell varient . Site: metaphysis of long bones – distal femur, proximal tibia (60%) flat bones – ileum, ribs, skull bone hand bones. Age – ranges from 3-70 years , 60% are late adolescents and young adults. Sex – M>F

33. X - RAY- Sharply marginated , lobulated,eccentrically located lucent defect in the metaphysis , may extend into epiphysis. Macroscopy : sharp circumscribed , often lobulated , firm, gray white or blue gray. Microscopy: pseudolobular architecture, spindle shaped or stellate cells, abondant myxoid stroma to chondroid stroma .

34. TREATMENT: Intralesional curettage or resection. Resulting bony defect has to filled using iliac bone graft and screw fixation. Recurrence rate 25%. Wide en block excision may lower the recurrence, but add unnecessary morbidity. Local adjunct treatment agents, such as phenol, methylmethacrylate and liquid nitrogen have not been shown to decrease the recurrence rate. Radiotherapy may be used in tumors that are considered unresectable .

35. CHONDROSARCOMA A chondrosarcoma is a malignant tumor , may develop from an enchondroma or osteochondroma but occurs more commonly in bones with no known preceding cartilage lesion. AGE- Chondrosarcomas occur in adults and the elderly. SITE- most commonly in the pelvis, scapula, humerus , femur, and tibia. Patients with multiple hereditary exostoses , Ollier's disease and Maffucci's syndrome have a higher risk for malignant transformation of a cartilaginous lesion. Chondrosarcomas vary considerably in their behavior : some enlarge rapidly, aggressively invade normal tissue, metastasize, and cause death, but most are low grade tumors that enlarge slowly, cause little damage to adjacent tissues, and metastasize only after many years.

36. Plain radiographs reveal bone destruction as well as mineralization within the tumor . HISTOPATHO: Chondrosarcoma of low grade may mimic Chondromyxoid fibroma except for the lack of a myxoid element. Low grade chondrosarcoma : few mitotic figures with a bland histologic appearance, enlarged chondrocytes with plump multinucleated lacunae. High grade chondrosarcoma : hypercellular stroma consisting of charecteristic “blue balls” of a cartilage lesion which permeate the bone trabeculae .

37. Wide surgical excision is necessary and offers the best possibility of cure, as radiation and chemotherapy have not proven to be effective methods of treatment for chondrosarcoma . Locally recurrent disease is common and may be difficult to treat.

38. THANK YOU