Journal of Indira Gandhi Institute Of Medical Sciences

ORIGINAL ARTICLE
Year
: 2021  |  Volume : 7  |  Issue : 1  |  Page : 12--16

Rare sites of giant cell tumor: Institutional experience


Indrajeet Kumar1, Wasim Ahmed1, Rahul Kumar1, Sanjeet Kumar2, Nishant Kashyap1, Santosh Kumar1, Manish Kumar3,  
1 Department of Orthopedics, IGIMS, Patna, Bihar, India
2 Department of Pathology, IGIMS, Patna, Bihar, India
3 Department of Surgical Oncology, SCI, IGIMS, Patna, Bihar, India

Correspondence Address:
Manish Kumar
Department of Surgical Oncology, SCI IGIMS, Patna 14, Bihar
India

Abstract

Introduction: Giant cell tumor (GCT) is a primary benign neoplasm of the bone. It accounts for 5% of all skeletal tumors. It usually originated from the long bones. It usually arises in the metaphyseal-epiphyseal area of the long bones. We report some cases of GCT at rare sites. These are clavicle, phalanx, head of fibula, distal end of the ulna, and extensor tendon sheath of the hand. Materials and Methods: All cases were investigated and managed in the Department of Orthopedics and Surgical Oncology, IGIMS, Patna, India, between March 2014 and June 2019. We surgically managed eight rare cases (5 male and 3 female) of GCT. Patients were followed up for minimum 1 year. Discussion: GCT represents about 3%–5% of all bone tumors and 21% of benign bone tumors. It is most commonly seen in early adulthood, with a peak incidence in the third decade and with a slight female preponderance; it is usually seen in the skeletally mature patient. Conclusion: GCT s of bone at rare sites present a challenge in both diagnosis and treatment because there is very few literature about them. Our study on this tumor at rare sites will throw a new insight into the proper management.



How to cite this article:
Kumar I, Ahmed W, Kumar R, Kumar S, Kashyap N, Kumar S, Kumar M. Rare sites of giant cell tumor: Institutional experience.J Indira Gandhi Inst Med Sci 2021;7:12-16


How to cite this URL:
Kumar I, Ahmed W, Kumar R, Kumar S, Kashyap N, Kumar S, Kumar M. Rare sites of giant cell tumor: Institutional experience. J Indira Gandhi Inst Med Sci [serial online] 2021 [cited 2021 Oct 26 ];7:12-16
Available from: http://www.jigims.co.in/text.asp?2021/7/1/12/318921


Full Text



 Introduction



Giant cell tumor (GCT) is a primary benign neoplasm of the bone. It accounts for 5% of all skeletal tumors. It usually originated from the long bones. It usually arises in the metaphyseal-epiphyseal area of the long bones. It is usually seen in the third and fourth decade with a slight female preponderance. About 50% of cases originate around the knee. We report some cases of GCT at rare sites. These are clavicle, phalanx, head of fibula, and distal end of the ulna. Occasionally, a GCT will be seen in a child.

 Materials and Methods



All cases were investigated and managed in the Department of Orthopedics and Surgical Oncology, IGIMS, Patna, India, between March 2014 and June 2019. We surgically managed eight rare cases (5 male and 3 female) of GCT [Table 1]. Patients were followedup for minimum 1 year.{Table 1}

Case no 1

A 8-year-old girl was admitted with a 1-year history of progressively increasing swelling and pain over the right lateral third of the clavicle [Figure 1]. There was no history of trauma. Pain was insidious in onset, dull aching in character, nonradiating, and localized to the lateral half of the clavicle without diurnal variations. Pain aggravated on the movement of the shoulder. A 5 cm × 3 cm bony mass arising from the clavicle at the junction of middle and lateral third which was globular in shape and hard in consistency. The overlying skin was mobile and free. The arm abduction was painful. There was no sensory or power loss in the left upper limb. X-ray revealed expansile radiolucent lesion without any periosteal reaction, soft-tissue component, or calcification. Computerized tomography showed lytic lesion arising from the right clavicle with areas of necrosis. Fine-needle aspiration cytology (FNAC) suggested the diagnosis of GCT. Provisional diagnosis of GCT was made and partial claviclectomy was done. Grossly, the tumor is 5 cm × 4 cm × 3 cm. The cut surface is solid and tan or light brown admixed with area of hemorrhage. Microscopically, tumor composed of stromal cells and giant cells. The giant cells are large, have twenty or more nuclei, most of them arranged toward the center, suggestive of GCT. Histopathological (HPE) examination confirmed the diagnosis. The postoperative period was uneventful. At 4-year follow-up, there was no disability or local recurrence or distant metastasis.{Figure 1}

Case no 2

A 19-year-old male presented with a complaint of pain and swelling over the outer aspect of the right knee for the past 1 year [Figure 2]. Onset was insidious and gradually progressive. Clinical examination revealed diffuse swelling at the anterolateral border of the right knee with normal knee movements and intact neurovascular status with full ankle range of motion. X-ray of the right knee with the leg in the anteroposterior and lateral views was done. It showed a well-defined osteolytic lesion in the epiphysio-metaphysial region of right proximal fibula without intra-articular extension. Magnetic resonance imaging (MRI) and FNAC were performed which suggested GCT. Excision of fibular head was done with 3 cm margin of the normal bone. MRI findings and FNAC finding were confirmed with bone biopsy. At 1-year follow-up, there was no disability or local recurrence or distal metastasis seen.{Figure 2}

Case no 3 and 4

There were two patients who one 26-year-old male and another 34-year-old male, presented with complaints of pain and swelling around the inner side of the left wrist [Figure 3]. Pain progressively increased in 4 months after the onset without any particular event. First one has swelling of 4 cm × 3 cm and another one with swelling of 3 cm × 2.5 cm in the distal end of the ulna. Overlying skin was normal and adhered to underlying swelling. The swellings were diffusely tender and hard. The range of motion of the left wrist was restricted without any neurovascular deficit. Moderate movemental pain was present at the extremes of movement in all directions. Routine blood examinations were within the normal limits. Plain X-ray of the wrist anteroposterior and lateral view showed an expansile, multilobular, and radiolucent lesion in the left ulna with the absence of periosteal reaction. Chest X-ray PA view showed no abnormality. FNAC suggested GCT. Wide local excision of tumors with adequate margins were done and sent for HPE examination which confirmed the diagnosis of GCT. Patients are in follow-up till date, which is more than 1 year, and at present, there is no recurrence or abnormality.{Figure 3}

Case no 5

A 47-year-old female patient presented with pain and swelling of the left hand on the ulnar side for 6 months without any history of trauma or constitutional symptoms [Figure 4]. On examination, a fusiform swelling in the fifth metacarpal region was noted, which was tender and firm in consistency, and had normal overlying skin without any scar or adherence to the underlying swelling. The adjacent joints had the normal range of movements. Radiograph showed an expansile lytic lesion involving the fifth metacarpal bone with no sign of periosteal reaction with intact articular margins. The chest radiograph was normal, and the laboratory investigations were within the normal limit. FNAC suggested GCT. Extended curettage using liquid phenol with bone grafting was done. At 1-year follow-up, there was no disability or local recurrence present.{Figure 4}

Case no 6, 7, and 8

There were 3 patients which have GCT of phalanges at different locations [Figure 5] and [Figure 6]. The diagnosis was confirmed by the cytological examination supported by radiological investigations.{Figure 5}{Figure 6}

First one a 18-year-old male patient presented with pain and swelling of the left middle finger base of middle phalanx for 5 months without any history of trauma or constitutional symptoms. On examination, a fusiform swelling in the middle phalanx of the left middle finger was noted, which was tender, firm in consistency, and had normal overlying skin without any scar or adherence to the underlying tissue. The adjacent metacarpophalangeal (MCP) and proximal interphalangeal joints had normal range of movements. Radiograph showed an expansile lytic lesion involving the base and proximal half of the middle phalanx shaft with a thin cortical rim. There was no sign of periosteal reaction with intact articular margins. The chest radiograph was normal, and the laboratory investigations were within the normal limit. FNAC suggested GCT. Extended curettage using liquid phenol with bone grafting was done. At 1-year follow-up, there was no disability or local recurrence present.

Second one 50-year-old male patient presented with pain and swelling of the left little finger above the MCP joint for 6 months without any history of trauma or constitutional symptoms. On examination, a fusiform swelling in the proximal phalanx left little finger region was noted, which was nontender and firm in consistency, and had normal overlying skin without any scar or adherence to the underlying swelling. The adjacent joints had normal range of movements. Radiograph showed an expansile lytic lesion involving the proximal fifth phalanx with no sign of periosteal reaction with intact articular margins. The chest radiograph was normal, and the laboratory investigations were within the normal limit. FNAC suggested GCT. Amputation of left little finger was done. At 3-year follow-up, there was no disability or local recurrence present.

Third one – 45-year-old female presented with swelling in index finger of the right hand with ulcer. She had a history of excision with reconstruction with fibula. It was a case of recurrence which was confirmed by the cytological and radiological examination. Second ray amputation was done. At present, there is no recurrence.

Summary of patient demographics, diagnosis, treatment, complication,

 Discussion



GCT represents about 3%–5% of all bone tumors and 21% of benign bone tumors.[1],[2] It is most commonly seen in early adulthood, with a peak incidence in the third decade with a slight female preponderance; it is usually seen in the skeletally mature patient.[3],[4] Giant cell tumor of the bone (GCTB) is generally considered a true neoplastic condition with well-defined clinical, radiological, and HPE features.[5] Radiologically, it is usually lytic and expansile without prominent peripheral sclerosis and periosteal reaction.[6] To reduce the high recurrence rate, the tumor can be treated with en bloc resection if the site is located in expandable bones such as proximal fibula, distal ulna, proximal radius, coccyx, and sacrum.[7],[8],[9]

The clavicle is a rare site for bone tumors.[10] Because there are only a few reports of clavicular GCTs,[3],[11],[12],[13] the optimal surgical method has not reached a consensus. However, it seems that claviculectomy, either partial or total, might be a good option for clavicular GCTs.[14] Krishnan et al.[15] reported that the postoperative function of the affected limb was normal and that only mild pain was present after total claviculectomy. In contrast, Rockwood and Wirth[16] reported unsatisfactory outcomes in most cases (85%) because of pain, loss of muscle strength, and shoulder drooping with or without neurovascular compression or shoulder joint instability; therefore, they recommended that surgeons preserve as much of the clavicle as possible.

The distal end of the ulna is an unusual site for a primary bone GCT.[17] This occurs in only 0.45%–3.2% of all primary bone GCT's.[17] Cooney et al. reported good functional outcome after distal ulna resection without any stabilization and concluded that reconstruction is not routinely indicated.[18]

Only 2% of all reported GCTs are found in the hand, with phalangeal bones being a very rare primary site of involvement. In reported cases, GCTs at a phalanx have shown higher recurrence rate as compared to those at more common locations like the distal femur. Local recurrence following simple curettage and bone grafting has been reported to be as high as 90%.[18] Curettage, simple or with an adjuvant like phenol or cryotherapy, whether in isolation or associated with bone graft, is the most common form of treatment, but its rate of recurrence reaches around 20%–90%.[19],[20] In cases of recurrence, a second similar local intralesional procedure is typically sufficient in cases that are detected early.[21]

 Conclusion



We have reported all these cases to emphasize that the diagnosis of GCTB may be missed at rare sites, both clinically and radiologically, unless there is a high index of suspicion. The presence of an expansile lytic lesion of the bone should be taken judicially and complete radiological and HPE investigation should be done with primary treatment being surgical. GCTB should be kept in mind, despite its rarity, for any expansile lytic lesion. The surgical treatment of these tumors differs as per their location and extent of lytic changes in the bone.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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