Indian Journal of Plastic Surgery
An open access publication of Association of Plastic Surgeons of India
Users Online: 1828  
Home | Subscribe | Feedback | Login 
  Navigate here 
 Resource links
 »   Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
 »   Article in PDF (4,274 KB)
 »   Citation Manager
 »   Access Statistics
 »   Reader Comments
 »   Email Alert *
 »   Add to My List *
* Registration required (free)  
  In this article
 »  Abstract
 » Introduction
 » Summary
 »  References
 »  Article Figures
 »  Article Tables

 Article Access Statistics
    PDF Downloaded157    
    Comments [Add]    
    Cited by others 2    

Recommend this journal


 Table of Contents    
Year : 2011  |  Volume : 44  |  Issue : 2  |  Page : 337-347

Malignant tumours of the hand and wrist

1 Dr Paul Brand Centre for Hand Surgery and Peripheral Nerve Surgery, Christian Medical College Hospital, Vellore, Tamil Nadu, India
2 Department of Pathology, Christian Medical College Hospital, Vellore, Tamil Nadu, India

Date of Web Publication24-Sep-2011

Correspondence Address:
Binu P Thomas
Dr. Paul Brand Centre for Hand Surgery and Peripheral Nerve Surgery, Christian Medical College Hospital, Vellore, Tamil Nadu 632 004
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-0358.85355

Rights and Permissions

 » Abstract 

Malignant tumours are rare in the hand and wrist. The clinical presentation may be similar to that of a benign lesion and a high index of suspicion is necessary so that such lesions are not missed by the treating surgeon. Out of a total of 657 tumours/tumour-like lesions of the hand and wrist seen in a tertiary referral centre in a 10-year period, a total of 39 tumours were identified as malignant (5.9%) and of which majority had origin from the skin (53.8%). The management of these tumours is primarily surgical. Limb salvage surgery may be applied when appropriate, though eradication of disease should be the primary goal rather than preservation of function. A multimodal approach is necessary for appropriate management including chemotherapy and radiotherapy.

Keywords: Hand tumours; malignant hand tumours; malignant tumour

How to cite this article:
Thomas BP, Sasi K, Pallapati SC, Mathew A, Sreekanth R, Thomas M. Malignant tumours of the hand and wrist. Indian J Plast Surg 2011;44:337-47

How to cite this URL:
Thomas BP, Sasi K, Pallapati SC, Mathew A, Sreekanth R, Thomas M. Malignant tumours of the hand and wrist. Indian J Plast Surg [serial online] 2011 [cited 2019 Aug 18];44:337-47. Available from:

 » Introduction Top

Tumours and tumour-like swellings in hand and wrist are mostly benign. Malignant tumours of the hand and wrist are uncommon and many of the hand surgeons see very few of these in clinical practice. Their initial behavior may be similar to that of a benign lesion and hence maybe missed with disastrous consequence for the patient. The common malignant tumours are tabulated in [Table 1] based on tissue of origin. It is imperative to understand the principles of staging, biopsy, resection and amputation before treating malignant tumours of the hand. Management protocols from literature in management of tumours are conflicting and contradictory. These protocols require different specialties coming together to attain the goal of tumour-free survival for the patient. Limb salvage surgery has become commonplace with refinements in chemotherapy, radiation therapy and reconstructive microsurgery. Though there exists multimodal management of these tumours, it must be stressed that current advances must be followed in treating the tumours of hand.
Table 1: Malignant tumours of hand and wrist

Click here to view

In India, patients generally tend to present later for treatment and hence may have more obvious disease. There have been no previous reports of the incidence and presentation of malignant tumours of the hand from an Indian perspective. In this article, we have reviewed the malignant tumours of the hand presenting at our tertiary referral specialised hand surgery centre. The various histological diagnosis of the patients seen between 2000 and 2010 are given [Table 2].The general management and brief description of common malignancy are also discussed.
Table 2: Histologic diagnosis and patients

Click here to view

Clinical features

Malignant tumours of the hand present generally as a painless swelling, present over many years, which may suddenly increase in size and become painful, or as a long-standing skin lesion/ulcer. Pathological fractures with minimal antecedent trauma may at times be the first presenting feature. Systemic features like fever may be present occasionally. Burn scars are known to develop malignant changes (Marjolin's ulcers). Subungual malignant lesions mimic chronic paronychia. Any chronic infective nail bed lesions not responding to usual management must be biopsied to rule out malignancy. Pigmented lesions in the nail bed must be viewed with suspicion and change in size of such lesion warrants a biopsy to rule out melanomas. Epitrochlear and axillary lymph nodes must be routinely checked. Carcinomas and malignant melanomas have a predilection to spread via lymphatics. A general examination to rule out liver involvement also must not be forgotten.

Out of a total of 657 tumours/tumour-like lesions of the hand seen in Dr Paul Brand centre for Hand Surgery which is a tertiary referral centre between years 2000 and 2010, a total of 39 tumours were identified as malignant (5.9%). The different tumour types, management and follow-up are given in [Table 3]. Of the 39, fifteen were skin malignancies (53.8%). Mean age is 37.31 (range 1 year(5 months) -67years). Male:female ratio is 2.9:1
Table 3: Guidelines for evaluation of hand tumours

Click here to view


Preliminary radiological study by X-rays is mandatory in evaluation of bony lesions. CT scans provide multiplanar evaluation of bony lesions. An MRI is of value in evaluation of soft tissue tumours to assess the extent of the tumour and relation to vital structures. Malignant lesions are generally enhanced on a T2 weighted and STIR images.

Other investigative modalities done as a routine for "tumour work up" includes a complete blood work up, chest X ray, CT/ USG of abdomen and Tc 199 bone scan [Table 3] . PET scans are somewhat helpful to assess the malignant potential of lesions and tumour response to chemotherapy.

Biopsy of tumours

Following the tumour work up, a biopsy must be done to establish/ confirm the diagnosis. The planning and execution of the biopsy is important and is best done by experienced tumour surgeons in centres where multimodal tumour management is routinely done. [1] The help of an experienced pathologist is very important in providing the right diagnosis on biopsy. Special stains and tumour markers help in establishing the correct cell lines. The approach must be carefully planned as the incision track will need to be excised during definitive surgery. The biopsy can be done using closed or open techniques. The biopsy technique used must provide adequate sample and information for accurate diagnosis and lead on to definitive care. Closed techniques include fine needle aspiration cytology (FNAC) and needle biopsy. The needle biopsy (commonly using a Trucut needle) yields adequate tissue for histopathologic examination and for evaluation of markers and special stains. Open techniques are done when the tumour is deep seated or when involving the bone. Incisional biopsies if required must be planned so that the path to the tumour can be completely removed when definitive surgery is done. Excisional biopsies are done for tumours <3 cm in size with an additional margin of 3 cm round the tumour also removed. Frozen section biopsies are useful in identifying whether a soft tissue lesion is malignant or not during definitive tumour surgery.

Evaluation and staging of malignant tumours

Staging helps in identifying tumour behavior, map the anatomic extent, propose appropriate treatment and helps to postulate prognosis. Enneking et al staged malignant tumours of bone based on histology, compartment and metastasis [2] [Table 4] and has been accepted by the Musculoskeletal Tumour Society (MSTS). The American Joint Committee on Cancer (AJCC) has proposed a staging system for soft tissue sarcomas where size of the lesion is also considered as important [3] [Table 5]. The AJCC staging system incorporates grade, size, depth, and the presence or absence of metastasis to determine the final stage of the lesion. This system should be used preferentially for staging of soft tissue sarcomas. The most important factor determining prognosis of the tumour is the histologic grading. [4]
Table 4: Enneking staging of malignant tumours of bone

Click here to view
Table 5: AJCC staging of soft tissue sarcomas

Click here to view

General principles of surgical treatment

In malignancies of the hand, complete eradication of the disease should be the primary goal rather than the preservation of function. [5] Enneking has grouped tumour surgery based on the tumour margins [Table 6]. Due to anatomical peculiarities unlike elsewhere, most hand tumours occur in spaces rather than in compartments. Compartmental resection and radical excision cannot be applied to the hand in its entirety. [6] Due to proximity of vital structures in the hand, wide resection retaining function, as practiced in case of tumours in other parts of the body is not always feasible in the hand. Similarly, compartment barriers are often breached quickly as muscles and neurovascular structures pass through "anatomic compartments" and makes compartmental resection difficult especially since tumour spreads through these structures.
Table 6: Surgical procedure for extremity sarcomas (Enneking)

Click here to view

Once the diagnosis is confirmed, a wide excision (also referred as an en bloc resection)) is planned where the tumour together with its pseudocapsule (reactive zone) and 2-3 cm of surrounding margin of normal tissue is removed. The whole muscle or entire bone is not removed in a wide excision. A radical resection in the hand involves removal of the tumour, its pseudocapsule and all involved muscle and bone as a single tissue block such as a digit or ray amputation. In case of a metacarpal tumour, excision of the metacarpal may remove a compartment for a stage IA or IIA bone tumour. However for a tumour arising from the flexor tendon of a digit, according to Enneking criteria, it would be required to remove the whole flexor surface or an above-elbow amputation to ensure radical resection if Enneking's guidelines are followed. This is neither feasible nor routinely done in case of hand lesions. Presently there is no comprehensive data to support a conclusion that the Enneking criteria must be applied uniformly to all parts of the hand. A soft tissue tumour that develops along the dorsum of the wrist requires surgical extirpation of the extensors of several fingers and/or the thumb but not necessarily of the entire muscle compartment proximally or distally. [7]

Amputation in hand malignancies

There is a definite role for amputations in the hand for malignant tumours. In general, the main goals of oncologic amputation surgery in the upper extremity are (1) obtaining clear margins, (2) preserving functional length, (3) preventing joint contractures, (4) prevention of painful neuromas, (5) facilitate early prosthetic fitting if appropriate, and (6) minimizing morbidity. [8] The level of amputation is dependent on the site of the tumour. A general scheme according to site of lesion is depicted in Diagram 1-[Additional file 1]. Depending on the level of the tumour, disarticulation at DIP, MCP, ray amputation (single or multiple), wrist disarticulation and below elbow amputation may be done. All basic rules of proper amputations must be adhered to.

Limb salvage in hand tumours

Whenever limb salvage is considered, it must be remembered that "compromising the resection margins out of concern for reconstruction will increase the chance of local recurrence, and may increase the risk of metastasis and ultimately death". [7] Advances in imaging and multimodal management of tumours together with newer techniques in microsurgery have helped in considering limb salvage surgery in the management of hand malignancies. Most of these procedures require microsurgical expertise and require a team effort by the tumour surgeon, hand surgeon and microsurgeon. Reconstruction is worthwhile if an adequate margin of resection can spare vital structures to provide a functioning end organ as no prosthesis can replace a functioning hand in terms of both prehension and sensibility. Principal arteries can be bridged by vein grafts to retain adequate perfusion in case a radical resection removes a segment of the vasculature. Similarly nerve defects can be bridged by sural nerve grafts with predictable return of function. Tendon transfers can be considered if a functional result is not possible by nerve grafting. Soft tissue defects following tumour resection may be closed primarily if possible or by local, regional or preferably free flaps. The specific flap to be used for a post resection defect depends on the size of the defect and location. Large dead spaces can be filled by muscle flaps. Smaller defects are amenable to local transposition or regional flaps, though as a rule, a distant or free flap is preferable to avoid further seeding of the tumour accidentally to neighbouring areas. The posterior interosseous artery flap and the lateral arm free flap are good options in the management of medium-sized defects of the hand. Bony tumours especially when confined within osseous boundaries when excised with adequate margin can be made good by autogenous nonvascularised or vascularised bone grafts. It is advisable to use vascularised bone grafts such as fibula when bony defects >6 cm need to be bridged. [9],[10] Osteocutaneous flaps such as fibula and iliac crest can address both skin and bone defect following resection.

Multimodality management in hand tumours

Adequate data is still unavailable regarding the indication and benefits of chemotherapy and radiation therapy in hand malignancies. The management of soft tissue sarcoma has evolved from a standalone surgery to a multidisciplinary approach. This change is the result of increased knowledge in tumour biology, radiation sensitivity and the improvement in modern radiation therapy techniques. A successful effective therapy regimen strongly depends on distinct preoperative diagnostics, preoperative conception of the surgical intervention and an experienced oncological team. Of prognostic significance is early diagnosis as well as tumour excision with a wide negative margin. [11] However, even after complete wide resection, the use of postoperative radiotherapy can further improve local control and should therefore be applied to the majority of patients. [11],[12],[[13] Consequently, radiotherapy should only be omitted in cases in which the tumour has been excised with a very wide negative margin; this implies, however, high quality of surgery and distinct histopathological analysis. Patients with non- or questionable resectable tumours should be referred for pre-operative radiotherapy in order to improve the surgical results. Recent studies have underlined the efficiency of modern radiotherapy regimens. [14]

Radiotherapy is also indicated if the lesion is unresectable or for reasons of medical inoperability. In these situations, definitive radiation can be given with potentially curative intent or palliation. [15] Soft tissue sarcomas have been found to be radiosensitive with a five-year local control reported to be about 33% and survival rate of 25%. [16] The number of patients undergoing adjuvant radiotherapy for soft tissue sarcomas is also on the rise. Patients with low-grade and high-grade lesions showed a significant reduction in local recurrence without change in overall survival. [12] External beam irradiation of 60-66 Gy is recommended for total post operative irradiation. [15] Preoperative external beam radiation therapy has the potential for reducing tumour size, minimize seeding, facilitating better results though increasing surgical wound problems due to delayed healing. In the hand radiation therapy can result in contractures, neuropathies and physeal growth disturbances with significant functional problems. We do not routinely carry out preoperative radiation in hand tumours.

Chemotherapy in hand malignancies

The role of chemotherapy in hand malignancies remains the same as in any other location. Adjuvant chemotherapy for soft tissue sarcomas remains controversial. For low-grade soft tissue sarcomas, due to poor sensitivity to chemotherapeutic agents, adjuvant chemotherapy is not recommended. In high-grade lesions the role of adjuvant chemotherapy may be promising, though randomised control trials have not corroborated this. [15] Conventional treatment options with surgery and radiation therapy for high grade large (>5 cm) and deep seated soft tissue sarcomas yield an approximate 50% three-year survival. [15] Tumours like Ewing's sarcoma/PNET and rhabdomyosarcoma definitely responds to chemotherapy. Recommended combination for this includes cyclophosphamide, vincristine and doxorubicin alternating with etopuside and ifosfamide. [15] The other commonly used chemotherapeutic agents include adriamycin, cisplatin, methotrexate and occasionally paclitaxel and carboplatin.

A brief description of the usual tumours along with our clinical experience with some of these are given below

Tumours of skin

In our series the cutaneous malignancies were the majority (21/39) or 53.8% which is similar in other reports. [17],[18] Most of these were squamous cell carcinomas SCC (15/21). Conventionally it is thought that Indians have fewer incidences of non melanoma skin cancers (NMSC) due to protective effects of melanin. However recent reports suggest that the incidence is on the rise especially in patients with dermatological conditions such as discoid lupus erythematosus, and lupus vulgaris. [19] The SCC in our series were associated with actinic keratosis, a premalignant condition resulting commonly from sun exposure and arsenic keratosis in four patients and Bowen's disease in two. [Figure 1] and [Figure 2] Arsenical keratosis is treated by topical 5- fluorouracil. Due to significant infected ulceration, lymphadenopathy is common. We give two weeks of antibiotics post operatively after tumour surgery and if lymph node size remains same, plan a lymph node biopsy or axillary clearance. Patient in [Figure 2]b had 4 I and Ds one for nailbed infection before biopsy showed a subungual SCC. Patient in [Figure 2]c developed axillary and systemic metastasis and succumbed within a period of eight months after presentation.

There were no basal cell carcinomas (BCC) in our series though it has been reported as the most common cutaneous malignancy with an incidence of less than 3% in the upper extremity. [20]
Figure 1: Squamous cell carcinoma (SCC)
(a) Infiltrating SCC left wrist, arsenical keratosis both hands. (b) Amputation left hand. (c) SCC right palm. (d) Excision and PPIA flap. (e) Hand E stain x100: nests of malignant squamous epithelial cells with keratin pearls

Click here to view
Figure 2 : Squamous cell carcinoma
(a) Squamous cell carcinoma from burn scar (Marjolins ulcer). (b) Subungual squamous cell carcinoma. (c,d,e) 20 year history of ulcer in the palm, four previous surgeries elsewhere, moderately differentiated SCC. Limited ray excision, epitrochlear lymph node removal, axillary clearance and local radiotherapy, three cycles of 60 Gy and chemotherapy with paclitaxel and carboplatin six cycles given

Click here to view

Malignant melanomas are pigmented lesions characterised by the ABCD rule: assymetric, border irregularity, colour variability, diameter greater than 6 mm. [21] Though reported to be rare in darker skinned population, it is not uncommon in India where typically patients present quite late. Subungual pigmented lesions should be viewed with suspicion and warrants biopsy. An excisional biopsy if possible is recommended. The most important pathologic determinant is the thickness using Breslow's technique. [22] A margin of 1 cm normal skin for each millimeter of Breslows tumour thickness is recommended (maximum margin 3 cm). [21],[23] An aggressive surgical excision should be done with sampling of the sentinel node to rule out lymph node metastasis. The patient in [Figure 3] developed axillary metastasis, after long standing thumb melanoma, had axillary clearance, refused further chemotherapy with dacarbazine as advised and died of pulmonary and brain metastasis.
Figure 3: Malignant melanoma (MM), (a) MM thumb, (b) Axillary metastasis, (c) High power(400x) view: dark brown granular melanin pigment within the tumour cells

Click here to view

Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade dermal sarcoma which extends into the subcutis, spread along fascial planes with a high incidence of recurrence and metastasis. They present as a hard nodule or a raised lesion. They can metastasize to lymph nodes or lungs. [17],[24] A wide excision (3 cm margin) along with removal of the underlying fascia [18] must be done. DFSP was seen in three patients. One patient had primary wide excision and skin closure. The other patient presented following three previous surgeries for a lesion over the dorsum of little finger with recurrence and required ray ablation [Figure 4].
Figure 4: Dermatofibrosarcoma protuberans (DFSP), (a,b) DFSP recurrence. Ray resection done. (c) Trucut biopsy specimen: characteristic diffuse CD34 cytoplasmic positivity on immunohistochemistry (d)

Click here to view

Soft tissue sarcomas

These are extremely uncommon and reports suggest that most upper extremity sarcomas are proximal to wrist. [7] They rarely spread to lymph nodes except for rhabdomyosarcoma, synoviosarcoma and epithelioid sarcoma.

Synoviosarcoma is a mesenchymal spindle cell tumour with variable epithelial differentiation and of biphasic nature (spindle and epithelial) on histology. [25] They are commonly seen to arise from the periarticular and synovial tissue. In the hand, it is usually seen around the wrist [Figure 5].
Figure 5: Synoviosarcoma, (a) swelling right thumb base, (b) B1, B2: X-ray and MRI showing the lesion with involvement of tendon sheaths and vascular structures, (c,d) A radical resection was done. (e) x200, Hand E staining showing sheets and nests of polygonal and spindle cells. (f,g) Epithelial membrane antigen EMA and CD99 positivity are shown

Click here to view
Diagnostic dilemmas exist and require an experienced pathologist to address. [26] It is generally positive for epithelial markers like cytokeratin or EMA and vimentin, BCl-2, CD99 and specific molecular PCR positivity. Wide excision with tumour free margins gives the best possible outcome. Role of radiotherapy and chemotherapy are debatable. [27]

Epitheloid sarcomas though rare are relatively common sarcomas of the hand seen in younger age group (15-35 years) with a male preponderance. They are extremely malignant and tend to spread along tendon sheath and fascial planes and lymphatics. [28] Radical resection or amputation is the best option for this aggressive tumour. Axillary node sampling /clearance is routinely done to identify spread. [Figure 6] shows a 36-year-old man who had a nodular lesion in the ring finger which became a nonhealing ulcer of many years duration. After biopsy, he had BE amputation followed by axillary clearance which was negative.
Figure 6: (a) Fibrosarcoma (FS), (a,b,c) Swelling 1st web space with MRI showing a well-defined rounded mass reported as schwannoma. Wide excision following needle biopsy reported as low-grade FS, (b) Epitheloid sarcoma (ES)

Click here to view

Myxoid chondrosarcoma is associated with the tendon sheath and usually seen in the digits. They are commonly seen in older men. [29] The patient in our series was a 24-year-old female with a lesion in the index finger of two years duration [Figure 7]. She underwent a ray ablation followed by axillary node clearance which was negative for the tumour.
Figure 7: Extraskeletal myxoid chondrosarcoma (ESMCS), (a,b) 24-year-old lady presented with mass, index finger, (c) X-ray: Expansile soft tissue mass with some lysis of the proximal phalanx, (d) Biopsy ESMCS underwent a ray resection, (e,f) Excellent function and cosmesis and is still being followed up after four years

Click here to view

Leiomyosarcoma arise from smooth muscle cells associated with blood vessels or sweat glands. They may be mistaken for a ganglion and hence it is important to consider this differential diagnosis in soft tissue swellings of hand and wrist. [30],[31] Metastasis to the lung is not uncommon due to its proximity to vascular structures. [18] In our series, a 16-year-old girl presented with a dorsal soft tissue mass and boutonniere deformity of the index finger was diagnosed as leiomyosarcoma and underwent a ray ablation after initial marginal resection and biopsy. No recurrence occurred on follow up of three years.

Unclassifiable tumours sometimes, fortunatey rarely, tumours resist classifying into any particular tissue type inspite of subjecting them to an array of special stains and immunohistochemical methods that we are forced to group them as malignant spindle cell spindle cell tumour [Figure 8].
Figure 8: Malignant spindle cell tumour (MSCT), (a,b) Five-month-old baby with four months history of swelling in the thumb, biopsy showed a MSCT with D/d infantile fibrosarcoma/rhabdomyosarcoma/leiomyosarcoma. Child underwent below elbow amputation. Post op chemotherapy with vincristine and D-actinomycin. No further follow up and status unknown. (c,d) Hand E showing sheets and fascicles of spindle-shaped cells with focal herring bone pattern. This spindle cell sarcoma shows focal positivity for desmin and smooth muscle actin (SMA)

Click here to view

Tumours of bone

Chondrosarcoma is the most common bony tumour of the hand and is usually seen as secondary chondrosarcoma in older age group (40-60 years) arising from a pre-existing cartilaginous lesion such as Ollier's disease or Maffucci syndrome. The onset of malignant transformation is usually with pain in a previously painless hard swelling along with progressive increase in size. These are slow growing tumours with good prognosis and have a recurrence rate of <10%. [18] Radical or wide excision by surgery is the treatment. They show poor response to radiation and chemotherapy.

Ewing's sarcoma/ PNET (primitive neuroectodermal tumour) classically presents mimicking an acute infection with pain, swelling and erythema in the affected area with associated leucocytosis and raised ESR. This is commonly seen in younger patients [Figure 9],[Figure 10]. Osteolytic lesions with classic "onion peel" appearance is described in most long bones while in the hand the lesion is commonly osteosclerotic or mixed lesion is seen on X-ray involving the phalanges. Three patients were diagnosed to have ES/PNET in our series, the youngest being 16 months old and the oldest 28. In the 16-month-old child [Figure 9] with four-month history of swelling of middle finger(a), X-ray showed a mixed sclerotic lesion with lytic areas involving the proximal phalanx (b). He was treated by ray resection (c and d). Child developed scapular lesion and chemotherapy using VIDE regimen (vincristine, ifosfamide doxorubicin and etopuside) was given with subsidence of scapular lesion. Three-year follow up shows no further recurrence of the tumour Osteosarcoma (OS), despite being the most common primary malignant bone tumour in adolescents and younger age group, is extremely rare in the hand. In the hand it is seen secondary to radiation, Pagets disease or as part of multicentric OS in patients with a median age of 50 years. [18] We did not come across this lesion in our series. Reports suggest that chemotherapy may not be necessary when this lesion is seen in the hand. [32]
Figure 9: Ewings sarcoma/PNET

Click here to view
Figure 10: Ewing's sarcoma/PNET, (a,b) 17-year-old boy with swelling and pain of the middle finger of one year, (c) X-rays: Expansile, sclerotic lesion of P1, (d) Ray excision followed by six cycles of vincristine, actinomycin, cyclophosphamide, (e,f) Follow up bone scans revealed a lytic lesion in the 4th rib for which local radiotherapy was given. He remains disease free on a three-year follow up, (g) Hand E staining showing lobules of small round cells, with hyperchromatic nuclei and scant cytoplasm exhibiting increased mitotic activity, (h) Immunohistochemistry shows tumour cells are CD99 positive

Click here to view

Malignant giant cell tumour usually arises from a preexisting benign state and is very aggressive. Irradiation also can bring about a malignant transformation in preexisting benign lesions. Metastasis can occur to the lungs. Surgical ablation is the best option. [33] Chemotherapy has been reported to be not effective. Local wide excision and reconstruction of distal radius with vascularised fibula grafting have been reported [34] in distal radius lesions. [Figure 11] shows a recurrent GCT with sarcomatous changes (A,B): This large fungating cauliflower growth with history of of swelling in the ring finger four years back which was treated elsewhere by amputation of ring finger had metastasis to the lung. She was treated by 4 th and 5 th ray amputation and chemotherapy with three cycles of doxorubicin. She had good local clearance, good function, but pulmonary lesions are progressing. . Distal radius is common for these tumours and recurrence of benign GCT does occur. [Figure 12] shows a reconstruction with vascularised fibula graft in a patient with recurrence of the GCT. A 30-year-old female with GCT distal radius initially treated elsewhere by excision and contralateral fibula grafting four years back and subsequently noticed swelling and progressive manus valgus deformity. B,C: X-ray and CT scan show recurrence with collapse of graft and involvement of proximal carpal row by tumour. D, E: Wide excision of tumour and graft, part of radius and proximal row carpectomy and distractor application to correct deformity and cement spacer was done. F, G,H,I: ipsilateral fibula harvested and vascularised fibula graft performed three months later with fusion of remaining carpus and 3 rd metacarpal to fibula.
Figure 11: Giant cell tumour with cellular atypia, GCT recurrence

Click here to view
Figure 12: Giant cell tumour recurrence

Click here to view

Metastatic disease is known for being extremely rare below the elbow. [35] Very few cases are available in the literature. We had a 36-year-old lady with adenocarcinoma lung who developed metastatic disease with lytic painful lesion in the proximal phalanx of the thumb [Figure 13]. Palliative chemotherapy for the primary disease included cisplatin, pemetrexed and docetaxel. However, she succumbed to the primary disease.
Figure 13: Secondaries in the hand, (a,b) X ray shows metastasis to thumb, (c,d) Patient with T cell lymphoma with cutaneous infiltration

Click here to view

Another patient with cutaneous lymphoma presented with severe infiltrations of tumour cells in both hands along with the skin over the rest of her body (mycosis fungoides).

 » Summary Top

Malignant tumours of the hand and wrist are rare. It is important therefore to identify these conditions as early as possible. Management of these tumours are primarily surgical aiming for complete clearance of the lesion along with a margin of normal tissue. Multimodality management of hand tumours is important and limb salvage surgery has a definite role.

 » References Top

1.Mankin HJ, Lange TA, Spanier SS. The hazards of biopsy in patients with malignant primary bone and soft-tissue tumors. J Bone Joint Surg Am 1982;64:1121-7.  Back to cited text no. 1
2.Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 1980;(153):106-20.  Back to cited text no. 2
3.Green FL, Page DL, Fleming ID, Fritz AG, Balch CM, Haller G, et al., AJCC cancer staging handbook, 6 th edition. New York: Springer-Verlag; 2002.p. 213-5.  Back to cited text no. 3
4.Wunder J. Musculoskeletal tumors: staging systems., in Orthopaedic knowledge update: musculoskeletal tumors. In: Menendez LR, editor.Rosemont; 2002. p. 21-7.  Back to cited text no. 4
5.Smith RJ. Tumors of the hand: who is best qualified to treat tumors of the hand? J Hand Surg Am 1977;2:251-2.  Back to cited text no. 5
6.Creighton JJ Jr, Peimer CA, Mindell ER, Boone DC, Karakousis CP, Douglass HO. Primary malignant tumors of the upper extremity: Retrospective analysis of one hundred twenty-six cases. J Hand Surg Am 1985;10:805-14.  Back to cited text no. 6
7.Athanasian EA. Bone and Soft tissue tumours, in Green's Text Book of Hand Surgery, H.R. Wolfe SW, Pederson WC, editor. Philadelphia: Elsevier; 2010.  Back to cited text no. 7
8.Tamurian RM, Gutow AP. Amputations of the hand and upper extremity in the management of malignant tumors. Hand Clin 2004;20:6, 213-20.  Back to cited text no. 8
9.Leit ME, Tomaino MM. Principles of limb salvage surgery of the upper extremity. Hand Clin 2004;20:5, 167-79.  Back to cited text no. 9
10.Yajima H, Tamai S, Ono H, Kizaki K, Yamauchi T, et al. Free vascularized fibula grafts in surgery of the upper limb. J Reconstr Microsurg 1999;15:515-21.  Back to cited text no. 10
11.Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, et al. The treatment of soft-tissue sarcomas of the extremities: prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 1982;196:305-15.  Back to cited text no. 11
12.Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998;16:197-203.  Back to cited text no. 12
13.Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF, et al. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 1996;14:859-68.  Back to cited text no. 13
14.Pape H, Orth K, Engers R, Matuschek C, Müller A, Hartmann KA, et al. [Radiotherapy of soft tissue sarcoma--part of a multidisciplinary strategy]. Wien Klin Wochenschr 2008;120:723-31.  Back to cited text no. 14
15.Maples WJ, Buskirk SJ. Multimodality treatment of upper extremity bone and soft tissue sarcomas. Hand Clin 2004;20:6, 221-5.  Back to cited text no. 15
16.Tepper JE, Suit HD. Radiation therapy alone for sarcoma of soft tissue. Cancer 1985;56:475-9.  Back to cited text no. 16
17.TerKonda SP, Perdikis G. Non-melanotic skin tumors of the upper extremity. Hand Clin 2004;20:293-301, 6.  Back to cited text no. 17
18.Plate AM, Steiner G, Posner MA. Malignant tumors of the hand and wrist. J Am Acad Orthop Surg 2006;14:680-92.  Back to cited text no. 18
19.Panda S. Nonmelanoma skin cancer in India: Current scenario. Indian J Dermatol 2010;55:373-8.  Back to cited text no. 19
20.Shanoff LB, Spira M, Hardy SB. Basal cell carcinoma: A statistical approach to rational management. Plast Reconstr Surg 1967;39:619-24.  Back to cited text no. 20
21.Perdikis G, TerKonda SP. Pigmented skin lesions of the upper extremity. Hand Clin 2004;20:283-91, 6.  Back to cited text no. 21
22.Breslow A. Thickness, cross-sectional areas and depth of invasion in the prognosis of cutaneous melanoma. Ann Surg 1970;172:902-8.  Back to cited text no. 22
23.Veronesi U, Cascinelli N, Adamus J, Balch C, Bandiera D, Barchuk A, et al. Thin stage I primary cutaneous malignant melanoma. Comparison of excision with margins of 1 or 3 cm. N Engl J Med 1988;318:1159-62.  Back to cited text no. 23
24.Jambhekar NA, Chinoy RC. Dermatofibrosarcoma protuberans--experience at a cancer hospital in India. Indian J Cancer 1988;25:94-103.  Back to cited text no. 24
25.Kottu R, Prayaga AK. Synovial sarcoma with relevant immunocytochemistry and special emphasis on the monophasic fibrous variant. J Cytol 2010;27:47-50.  Back to cited text no. 25
26.Kumar S, Chowdhury N. Accuracy, limitations and pitfalls in the diagnosis of soft tissue tumors by fine needle aspiration cytology. Indian J Pathol Microbiol 2007;50:42-5.  Back to cited text no. 26
[PUBMED]  Medknow Journal  
27.Okcu MF, Despa S, Choroszy M, Berrak SG, Cangir A, Jaffe N, et al. Synovial sarcoma in children and adolescents: Thirty three years of experience with multimodal therapy. Med Pediatr Oncol 2001;37:90-6.  Back to cited text no. 27
28.Peimer CA, Smith RJ, Sirota RL, Cohen BE. Epithelioid sarcoma of the hand and wrist: Patterns of extension. J Hand Surg Am 1977;2:275-82.  Back to cited text no. 28
29.Abramovici LC, Steiner GC, Bonar F. Myxoid chondrosarcoma of soft tissue and bone: A retrospective study of 11 cases. Hum Pathol 1995;26:1215-20.  Back to cited text no. 29
30.Brenton GE, Johnson DE, Eady JL. Leiomyosarcoma of the hand and wrist. Report of two cases. J Bone Joint Surg Am 1986;68:139-42.  Back to cited text no. 30
31.DeHart MM, Bowyer MW, Silenas R. Leiomyosarcoma of the skin and subcutaneous tissue of the hand and wrist. J Hand Surg Am 1992;17:481-3.  Back to cited text no. 31
32.Okada K, Wold LE, Beabout JW, Shives TC. Osteosarcoma of the hand. A clinicopathologic study of 12 cases. Cancer 1993;72:719-25.  Back to cited text no. 32
33.Rock MG, Sim FH, Unni KK, Witrak GA, Frassica FJ, Schray MF, et al. Secondary malignant giant-cell tumor of bone. Clinicopathological assessment of nineteen patients. J Bone Joint Surg Am 1986;68:1073-9.  Back to cited text no. 33
34.Pho RW. Malignant giant-cell tumor of the distal end of the radius treated by a free vascularized fibular transplant. J Bone Joint Surg Am 1981;63:877-84.  Back to cited text no. 34
35.Viswanathan PN, Rangad F, Roul RK. Metastases to the hand from carcinoma of the lower alveolus. J Hand Surg Br 1996;21:544-6.  Back to cited text no. 35


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]

This article has been cited by
1 Thumb reconstruction using a free osseocutaneous fibular flap following excision of an epithelioid haemangioendothelioma
Anderson, I.B. and Gilmour, A. and Rahman, K.M.A. and Sainsbury, D.C.G. and Ragbir, M. and Milner, R.H.
European Journal of Plastic Surgery. 2013; 36(6): 391-394
2 Thumb reconstruction using a free osseocutaneous fibular flap following excision of an epithelioid haemangioendothelioma
Iain B. Anderson,Adam Gilmour,Kazi M. A. Rahman,David C. G. Sainsbury,Maniram Ragbir,Richard H. Milner
European Journal of Plastic Surgery. 2013; 36(6): 391
[Pubmed] | [DOI]


Print this article  Email this article


Site Map  |  Home  |  Contact Us  |  Feedback  |  Copyright and Disclaimer
Online since 11th March '04
Published by Wolters Kluwer - Medknow