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MALIGNANT BONE AND

SOFT TISSUE TUMOURS

Christian Dumontier, MD, PhD


Centre de la Main, Guadeloupe, FWI
With the help of Caroline Leclercq, MD

To be downloaded at www.diuchirurgiemain.org
MALIGNANT HAND LESIONS
Skin Soft-tissue Bone
• Squamous cell • Rare (15% of 7000 new • Very Rare
carcinoma yearly cases in USA
involve the upper
• 75% of skin
• Chondrosarcoma
extremity)
cancers of the is the most
hand • Epitheloid sarcoma, frequent malignant
synovial sarcoma and bone tumour at
• 35-90% of Malignant Fibrous the hand
malignant hand histiocytoma are the most
tumours frequent at the hand
TAKE HOME MESSAGE
• Clinical presentation is highly variable ☞ be
suspicious ⚠

• If malignancy is suspected ☞ multidisciplinary


treatment plan

• Biopsy is the key for diagnosis 🔐


CLINICAL EXAMINATION
• Onset of symptoms

• Pain: Acute, progressive, Constant.


Location, radiation.

• Mass: location, associated


symptoms (nerves, ischemia,…)

• Others: swelling, fevers, night Synovial sarcoma

sweats, weight loss,…

• Family history (hereditary multiple


exostosis, Gardner, Maffuci, Olllier,
Neurofibromatosis,…)

Maffuci’s syndrome
IMAGING
Synovial sarcoma

• Plain X-rays with orthogonal


views

• Bone tumor ?

• Soft-tissue lesions may be


Chondrosarcoma
seen by changes in
shadowing

• Calcifications (synovial sarcoma,


liposarcoma, hemangioma, HO,
myositis ossificans, synovial
chondromatosis, tumoral calcinosis)
2ND LINE IMAGING
• US

• MRI with Gadolinium:

• Must include all the bone to


look for skip metastases.

• Bone marrow, soft tissue


structures, local edema, peri-
articular structures

• CT scan:

• Bony structures
Recurrent Giant Cell Tumour
IF MALIGNANCY SUSPECTED
• Chest, abdomen and pelvis CT

• Technetium bone scan / PET scan most often employed for searching metastases.

• Laboratory studies:

• Serum calcium, phosphorus, blood urea nitrogen, and creatinine (metabolic bone
disease ?).

• Serum alkaline phosphatase and lactate dehydrogenase (elevated in some


malignancies).

• Serum immunoelectrophoresis (multiple myeloma ?).

• Hematologic profile and ESR are abnormal in many neoplastic processes


INDICATIONS FOR BIOPSY
• Limited in hand as most lesions are benign and a pre-operative
diagnosis is almost certain

• « If the clinical and radiographic diagnosis of a bone


or soft-tissue tumor cannot be limited to benign
lesions only, biopsy must be strongly considered ».

• No need to emphasize how important the technique is !

Pradhan A, Cheung YC, Grimer RJ, Peake D, Al-Muderis OA, Thomas JM, et al. Soft-tissue sarcomas of the hand: oncological
outcome and prognostic factors. J Bone Joint Surg Br 2008;90:209–14.
Bray PW, Bell RS, Bowen CV, Davis A, O’Sullivan B. Limb salvage surgery and adjuvant radiotherapy for soft tissue sarcomas of the
forearm and hand. J Hand Surg Am 1997;22:495–503.
RISKS OF BIOPSY
• Adequate volume of tissue for microbiologic culture, pathologic analysis, and molecular
assays.

• Most important step is planning.

• Take care of suboptimal incision placement:

• Ideally longitudinal and readily incorporated in limb-limb-sparing incision

• Avoid Bruner-type (extensive soft-tissue contamination)

• Amputation needed in 18% of cases due to improper biopsy

• Excision biopsy/marginal excision results in large contamination including suture


placements, drain tracts, …only for high suspicion of benign lesion
Mankin HJ et al. The hazard of biopsy in patients with malignant primary bone and soft tissue tumors. JBJS Am 1982;64(8):
1121-1127.
Mankin HJ et al. The hazards of the biopsy, revisited. JBJS Am 1996;78(5):656-663.
TECHNIQUE OF BIOPSY
• Fine-needle aspiration (63-77% diagnostic accuracy)

• Core-needle biopsy: diagnostic accuracy 83-93%

• Incision biopsy: Diagnostic accuracy 94-98%

• Dissection performed directly to the tumor through planes

• Perfect hemostasis, sutures are close to the incision line;

• Draw the limb salvage incision or anticipated amputation flaps at the time of the
biopsy prior to drawing the biopsy incision.

• Wide excision should be oriented (short = proximal, long = lateral/radial is


conventional).

• Do not open the biopsy on the table (impair margin assessment).

• Avoid using contaminated instruments with hands. All instruments for biopsy are
removed, new drapes,…if another surgery is performed on the same patient.
PARTICULARITIES
• Nerve lesions: Marginal excision is preferred to biopsy sample

• Distal radius:

• Small lesion can be approached palmarely between radial artery and


1st dorsal compartment.

• Large lesions may need to be approached dorsally (be careful with


the EDC).

• Palm, Carpal tunnel, antecubital fossa: wide excision is recommended as


limited incision will contaminated all the area.
SENTINEL NODE BIOPSY
• Controversial as less than 5% of
sarcomas develop metastases to
lymph node

• May be indicated for soft-tissue


sarcomas with predilection for
lymphatic metastases
• Rhabdomyosarcoma, alveolar sarcoma,
clear cell sarcoma, epithelia sarcoma,
synovial sarcoma.
PATHOLOGY
• Type of cell American joint committee on cancer histologic grading system

• Cellular shape Grade Differentiation Designation


• Apparence with different GX Indeterminate undetermined
stains G1 Well-differentiated Low
G2 Moderate Intermediate
• Overall cellularity
G3 Poor High
• Atypia G4 Undifferrentiated High

• Mitotic figures ans other


By definition: Low grade < 15% chance of metastasis. High
nuclear characteristics grade > 15%
Based upon mitotic index, extent of spontaneous necrosis,
degree of tumor differentiation, cellularity, pleiomorphism.
MOLECULAR DIAGNOSIS
• Polymerase chain reaction Diagnosis Translocation
Alveolar rhabdomyosarcoma t(2;13)

• Fluosrecent in-situ Clear cell sarcoma t(12;22)

hybridization Dermatofibrosarcoma
t(17;22)
protuberanza

• Antibody-based assays Ewing’s sarcoma t(11;22)

Follicular t(14;18) Mantle cell


Lymphoma
t(11;14)

• Genetic translocations (can Myxoid chondrosarcoma t(9;22)

be pathognomonic for Myxoid liposarcoma t(12;16)

certains pathologies) Synovial sarcoma t(X;18)

Translocations associated with musculoskeletal tumors


STAGING
• Final step

• Enneking (Musculoskeletal tumor society) system is preferred for bone lesions

• Type of cell categories is the most important and classified as: Chondrogenic/
osteogenic/fibrogenic/fibrohistiocytic/hematopoietic/osteoclastic giant cell-rich/notochordal/vascular/
myogenic-lipogenic-epithelial/undefined + Ewing sarcoma

• American Joint committee on cancer staging system is preferred for soft tissue sarcomas (more predictive
of systemic relapse than MSTS)

• Type of cell or tissue is less important than

• Tumor depth refers to whether it is superficial or deep to the fascia

• Size of tumor is more important than its depth

• Presence/absence of metastasis carries the most significant prognostic weight


MUSCULOSKELETAL TUMOR SOCIETY
STAGING SYSTEM (ENNEKING)
Stage Histology Anatomic location Metastasis

Ia Low grade Intracompartmental Absent

Ib Low grade Extracompartmental Absent

IIa High grade Intracompartmental Absent

IIb High grade Extracompartmental Absent

Intra- or
III Low or High grade Present
extracompartmental
AMERICAN JOINT COMMITTEE ON
CANCER’ S STAGING SYSTEM
Stage TNM stage Histology

IA T1, N0, M0 Low grade

IB T2-3, N0, M0 Low grade

IIA T1, N0, M0 High grade

IIB T2, N0, M0 High grade

III T3, N0, M0 High grade

IVA T1-3, N1, M0-1b High grade

IVB T1-3, N0-1, M1b High grade


PRINCIPLES OF (SURGICAL) TREATMENT

• Save life before function

• If the hand is salvageable:

• Obtain negative surgical margins: Major functional issue is


nerve

• Then stabilize the limb and reconstruct to allow rapid


rehabilitation of the hand

• One stage procedure if possible +++


AMPUTATION

• Definite role

• Proximity of vital structures


makes wide resection
retaining function
impossible

• Schematically: Fingers are


amputated, forearm are
preserved

Tamurian RM, Gutow AP. Amputations of the hand and upper extremity in the management of malignant tumors. Hand Clin
2004;20:6, 213-20
IF THE HAND IS SALVAGEABLE
• Wide excision with 2 to 3 cm
of normal tissue margin

• Sacrifice of neurovascular
structures can be avoided,
provided that there is
meticulous attention to
dissection, a margin of normal
tissue can be obtained, and
neurovascular structures are
not surrounded by tumor.

Lazerges C. Soft tissue sarcomas of the forearm, wrist and hand. Hand Surgery and Rehabilitation 36 (2017) 233–243
IF HAND SALVAGE IS POSSIBLE: FOR BONE
DEFECT

• Prefer autologous bone (Allograft for smaller defect)

• Prefer internal fixation (should be stable)

• Prefer vascularized bone graft over 5 cm defect that allow for primary
bone healing in addition to creeping substitution ; reduce the risk of
fracture and infection, ability to remodel under stress.

• Vascularized fibula (union rate of 80% in first time and 97% following
supplemental bone grafting).

• Stress fracture are seen in 17% of patient at the forearm.


IF HAND SALVAGE IS POSSIBLE: FOR SKIN COVERAGE

• Prefer free flaps to pedicle flaps

• Contraindication to free flaps are hyper coagulable state, recent deep-


venous thrombosis, ongoing infection, inadequate recipient vessels

• Most used flaps: Radial forearm flap [(chinese) can include tendon, bone];
Posterior interosseous artery flap; Scapular and parascapular flaps; ALT
flap [can include bone, tendon, fascia,…]; Lateral arm flap

• Fasciocutaneous flaps are more versatile, more tolerant to radiotherapy


than muscle flaps [latissimus dorsii, serratus anterior, rectus abdominis (TRAM), gracilis
(free-functioning)]

• Not to forget the groin flap


OTHER TREATMENT MODALITIES:
RADIOTHERAPY

• If the lesion is unresectable or for reasons of medical inoperability.

• 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%.

• Patients with low-grade and high-grade lesions showed a significant


reduction in local recurrence without change in overall survival.

• Preoperative external beam radiation therapy is controversial



OTHER TREATMENT MODALITIES:
CHEMOTHERAPY

• Still under investigation.

• Quite efficient for osteosarcoma as an example.

• May have a small but real overall survival benefit


and may increase the disease-free interval in well-
selected patients with extremity lesions.
ISOLATED LIMB PERFUSION

• Consists of perfusing a limb (with


tourniquet) with tumor necrosis
factor (TNF-alpha) or Melphalan
a

• In locally very advanced STS


OUTCOMES
• Few series

• 5-year survival of 89% for distal STS relative to 66% for proximal STS;

• Recurrence-free 5-year survival was 87% versus 74%

• Survival without metastasis was 84% versus 66%

• Overall 5-year survival between 80% and 90%

• Inappropriate surgical margins lead to a 12-times higher risk of local


recurrence
Yamada Y, Nishida Y, Nakashima H, Sugiura H, Tsukushi S, Kamei Y, et al. Oncologic and functional outcomes of soft tissue sarcomas
of the distal upper extremity: comparison with those of the proximal upper extremity. Int Surg 2010;95:33–9.
Pradhan A, Cheung YC, Grimer RJ, Peake D, Al-Muderis OA, Thomas JM, et al. Soft-tissue sarcomas of the hand: oncological
outcome and prognostic factors. J Bone Joint Surg Br 2008;90:209–14.
CHONDROSARCOMA
• Most common primary malignant bone tumor in the
hand.

• May arise primarily or may develop from a preexistent


benign lesion

• Patients > 60 yrs old

• Proximal phalanges and metacarpals

• A slowly growing, firm, and often painful mass

• X-rays: tumor matrix with stippled calcifications, areas


of lysis, and poorly defined borders.

• Majority are low-grade slow-growing lesions that do


not metastasize. Risks of metastases is 10% usually after Evolution of a
local recurrence following intralesional treatment. chondrosarcoma
OSTEOGENIC SARCOMA
• Not children and adolescents at the hand, but in older patients in the fourth, fifth, and
sixth decades,

• Often arising from a preexistent lesion.

• Survival in patients has improved with the use of systemic chemotherapy, but this is
unclear at the hand

• No significant difference in survival with the use of chemotherapy before excision (neo-
adjuvant) or after excision (adjuvant),

• No role for external beam irradiation

• Long-term survival after aggressive treatment of local recurrence may be better for
lesions occurring in the hand than in general case series,
DERMATOFIBROSARCOMA
PROTUBERANS (DARIER-FERRAND)
• DFSP is a cutaneous malignancy that arises from the
dermis and invades deeper tissue

• DFSP is a locally aggressive tumor with a high recurrence


rate. Overall risk for the development of metastatic
disease is 5%, including 1% with regional lymph node
metastasis and 4% with distant metastasis

• DFSP most commonly occurs on the trunk (42-72%),


followed by the proximal extremities (16-30%). DFSP
rarely occurs above the neck (10-16%) - Case reports in
fingers

• Usually occurs in adults aged 20-50 years (extremes


newborns - 80 y)

• Surgical excision remains the mainstay of treatment for


DFSP.

• High local recurrence rate (up to 49% reported). Most


recurrences occur within 3 years of the primary excision
(mean 32 months)

For a complete presentation of that specific lesion, see www.diuchirurgiemain.org


HAND SOFT-TISSUE SARCOMAS
• In the web space, dorsum of the hand, palm, or wrist.

• Apart rhabdomyosarcomas (in children), soft tissue sarcomas appear in adults > 30.

• Three most prevalent histologic forms are: (1) epithelioid sarcoma, (2) synovial sarcoma, and (3)
malignant fibrous histiocytoma.

• High-grade, nonmetastatic sarcomas of the hand are very aggressive and have a 5-year survival
rate of 50% to 80%. This may be due to inadequate surgical excision, the anatomy of the hand,
or the intrinsic behavior specific to hand sarcomas

• Recent studies have shown that aggressive, limb-sparing surgery combined with adjuvant
therapy offers equivalent survival compared to hand amputation.

• Tumors distal to the MP joint are best treated with amputation.


Brien EW et al. Management of soft-tissue sarcomas of the hand. J Bone Joint Surg 1995;77A:564–71.
Lin PP et al. Surgical management of soft tissue sarcomas of the hand and foot. Cancer 2002;95:852–61.
Lazerges C. Soft tissue sarcomas of the forearm, wrist and hand. Hand Surgery and Rehabilitation 36 (2017) 233–243
HAND SARCOMAS
• Epithelioid sarcoma • Leiomyosarcoma

• Synovial sarcoma • Liposarcoma

• Malignant peripheral nerve


• Malignant histiocytofibroma sheath tumor
(undifferentiated pleomorphic
sarcoma) • Dermatofibrosarcoma
protuberans
• Clear-cell sarcoma
• Myxoinflammatory fibroblastic
sarcoma
• Fibrosarcoma •
EPITHELIOID SARCOMA
Epitheloid sarcoma in a 32 years old lady

• Most frequent at the hand

• Presents as one or more slow-growing, tan-white, palpable


nodules in the deep soft tissues, subcutis, or dermis. May have
an indistinct infiltrating margin, and ulceration is frequently seen.

• Most often on the fingers, hands, and forearms of males


between the ages of 15 and 35.

• A past history of injury is found in 25% of cases.

• Calcifications are seen on radiographs in 20% of cases

• High risk for local recurrence and metastasis (lymph nodes


(33%) and lung (50%) - Prophylactic lymphadenectomy is
recommended.

• Local recurrence rates 35 to 85%


SYNOVIAL SARCOMA
Pleiomorph sarcoma
METASTATIC TUMORS

You can downloaded a not yet published paper with a huge


literature review of 564 reported metastases in 482 patients by
Marieke Cattelan at www.diuchirurgiemain.org
CONCLUSION
• Clinical presentation is highly variable ☞ be
suspicious ⚠

• If malignancy is suspected ☞ multidisciplinary


treatment plan #

• Biopsy is the key for diagnosis 🔐