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Case Report|Articles in Press

A Rare Disease Presentation of Malignant Fungating Acral Melanoma of the Hand

Open AccessPublished:February 24, 2023DOI:https://doi.org/10.1016/j.jhsg.2023.01.015
      Malignant melanoma is relatively uncommon and accounts for 1%–3% of all malignant tumors. Malignant melanoma of the hand is exceptionally rare and highly malignant, with rapid progression if left untreated. Early clinical symptoms can be overlooked, and the tumor is often at a late stage when patients seek care, prompting amputation of the affected region. We present a case of a 48-year-old man who presented with a rapidly progressive, large, fungating mass of the distal aspect of the little finger diagnosed as malignant melanoma. We describe the presentation and treatment of this patient, who ultimately underwent partial amputation of the fifth metacarpal. Histologic analysis demonstrated nodular melanoma.

      Key words

      Fifteen percent of tumors of soft-tissue origin occur in the hand. The majority of these tumors are benign, which commonly include ganglion cysts, epidermal inclusion cysts, and giant-cell tumors of the tendon sheath.
      • Strike S.A.
      • Puhaindran M.E.
      Tumors of the hand and the wrist.
      Although malignant tumors of the hand, which mostly originate from the skin, soft tissue, or bone, are far less common than their benign counterparts, there is potentially a greater risk of disabled function and metastatic potential because of limited subcutaneous layer of the hand and close adjacency to important anatomic structures.
      • Chapman T.
      • Athanasian E.
      Malignant tumors of the hand.
      ,
      • Turner J.B.
      • Rinker B.
      Melanoma of the hand: current practice and new frontiers.
      Four percent of soft-tissue sarcomas arise in the hand. The most common soft-tissue sarcomas are epithelioid and synovial sarcomas. These should be considered as a differential diagnosis until ruled out when there is a painless hand mass with rapid growth and ulceration.
      • Strike S.A.
      • Puhaindran M.E.
      Tumors of the hand and the wrist.
      Because of overlapping characteristics shared by malignant and benign hand tumors, careful recording of history and physical examination should be performed, followed by radiographs, which may show cortical erosions. Magnetic resonance imaging may be useful in establishing a differential diagnosis in addition to visualizing tumor proximity to neurovascular or tendinous structures. If necessary for a definitive diagnosis, prompt referral to a cancer center for biopsy and staging may be performed.
      • Chapman T.
      • Athanasian E.
      Malignant tumors of the hand.
      Malignant melanoma is relatively uncommon and accounts for 1%–3% of all malignant tumors. Malignant melanoma of the hand, most commonly presenting on the palm, dorsum of the hand, and tip or nail complex following trauma, is exceptionally rare and highly malignant, with rapid progression if left untreated.
      • Turner J.B.
      • Rinker B.
      Melanoma of the hand: current practice and new frontiers.
      ,
      • Yang Z.
      • Xie L.
      • Huang Y.
      • et al.
      Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
      The most common forms of melanoma of the hand are the acral lentiginous and nodular subtypes of subungual melanoma. The differential diagnoses of pigmented nail lesions due to subungual melanoma include infection, hematoma, and chemical or medication exposure, among others.
      • Turner J.B.
      • Rinker B.
      Melanoma of the hand: current practice and new frontiers.
      Similar to more common enchondroma or chondrosarcoma, lytic lesions of the affected bone are often demonstrated on radiographs.
      • Chapman T.
      • Athanasian E.
      Malignant tumors of the hand.
      ,
      • Tomas X.
      • Conill C.
      • Combalia A.
      • Pomes J.
      • Castel T.
      • Nicolau C.
      Malignant melanoma with metastasis into the capitate.
      Although early diagnosis is crucial for malignant melanoma, early clinical symptoms can be overlooked, and the tumor is commonly at a late stage when patients seek care. Diagnosis is made using excisional biopsy for any cases that present clinically with areas of dark patches and uneven borders of the hand or finger.
      • Yang Z.
      • Xie L.
      • Huang Y.
      • et al.
      Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
      Axillary lymph node dissection is recommended for late-stage malignant melanoma affecting the hand.
      • Yang Z.
      • Xie L.
      • Huang Y.
      • et al.
      Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
      Chemotherapy and biologic therapy have historically shown low efficacy in the treatment of malignant melanoma, and the majority of patients undergo amputation for definitive treatment of the affected area.
      • Yang Z.
      • Xie L.
      • Huang Y.
      • et al.
      Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
      ,
      • Cho K.
      • Jung S.T.
      Treatment outcomes of advanced stage malignant melanoma in hand and foot after amputation in Korean patients.
      Because of the rarity of malignant melanoma of the hand, we present a case of a 48-year-old man with a nodular melanoma affecting the fifth distal phalanx. We describe the presentation and treatment of this patient, who ultimately underwent amputation of the fifth ray to the midshaft of the metacarpal. Written informed consent was obtained from the patient with regard to publishing of this report and associated figure images.

      Case Report

      A 48-year-old man initially presented to the emergency department with a foul-smelling, necrotic, fungating mass on his right little finger (Fig. 1). The patient reported trauma to this digit from a cactus needle 4 years prior to this initial visit to the emergency department. Irrigation and debridement of the digit were performed at this time, and cultures were taken, which revealed Pseudomonas aeruginosa and Enterococcus faecalis. After this initial visit, the patient was lost to follow-up for 4 months and presented to the emergency department a second time. The patient underwent radiography but refused all work-up at this time and left against medical advice to seek naturopathic treatment at home (Fig. 2). The patient presented to the emergency department again 6 months later seeking wide resection of the mass of the right little finger at the level of the metacarpal shaft (Fig. 3). The mass was black-brown and measured 10 × 7.5 × 6 cm3 (Fig. 4). Computed tomography of the chest yielded negative results for metastases. The patient refused additional work-up, including lymph node biopsy. The patient desired only amputation because of the inability to use gloves for work.
      Figure thumbnail gr1
      Figure 1A A clinical image of the right little finger at initial presentation. B Radiographs of the right hand, with the anteroposterior view, C oblique view, and D lateral view demonstrating a soft-tissue mass of the little finger, with evidence of lytic lesions at the distal phalanx. There was an incidental finding of a previously healed proximal phalangeal base fracture, which appeared healed.
      Figure thumbnail gr2
      Figure 2Radiographs of the right hand approximately 4 months from the initial presentation. A Anteroposterior view, B oblique view, and C lateral view demonstrating enlargement of the previous soft-tissue mass of the little finger, with evidence of lytic lesions at the distal phalanx and expansion into the middle phalanx.
      Figure thumbnail gr3
      Figure 3Radiographs of the right hand approximately 10 months from the initial presentation. A Anteroposterior view and B lateral view demonstrating further enlargement of the soft-tissue mass of the little finger, with expansion into the middle phalanx and head of the proximal phalanx.
      Figure thumbnail gr4
      Figure 4A clinical image of the right little finger at 10 months from the initial presentation. Gross images of the A dorsal surface and B volar surface.
      The patient underwent wide surgical excision of the mass. The large, fungating mass of the little finger was covered with a blue towel, and the tourniquet was inflated without exsanguination of the extremity, taking care to prevent any contamination from the mass. A curvilinear incision was made around the metacarpophalangeal joint of the small finger. Exploration revealed no obvious involvement of the proximal tissues or bone. Deep structures were sharply incised, and partial resection of the metacarpal was performed.
      The mass was sent for pathology, which revealed a malignant, nodular melanoma with clear, wide margins greater than 30 mm from the peripheral margin (Fig. 5). Postoperative radiographs demonstrated successful amputation of the little finger at the level of the metacarpal shaft (Fig. 6). After the procedure, the patient was lost to follow-up despite repeated attempts to reach out to him.
      Figure thumbnail gr5
      Figure 5Histology images of the mass of the right little finger. A Melanoma with skin ulceration (left) and focal heavy melanin pigmentation (lower right) (Hematoxylin-eosin stain; magnification × 12.5). B Melanoma cells, mainly spindle shaped, with the presence of dispersed melanin (Hematoxylin-eosin stain; magnification × 40). C Pleomorphic melanoma cells with melanin pigment (Hematoxylin-eosin stain; magnification × 100). D Brisk mitotic activity in melanoma (Hematoxylin-eosin stain; magnification × 400).
      Figure thumbnail gr6
      Figure 6Postoperative radiographs of the right hand, with the A anteroposterior view, B oblique view, and C lateral view demonstrating successful amputation at the fifth metacarpal midshaft.

      Discussion

      Malignant tumors of the hand can pose a challenge to hand surgeons because of the close proximity of important bony, tendinous, and neurovascular structures.
      • Strike S.A.
      • Puhaindran M.E.
      Tumors of the hand and the wrist.
      The reported malignant soft-tissue tumors of the hand include epithelioid and synovial sarcoma, myxofibrosarcoma, liposarcoma, rhabdomyosarcoma, and malignant peripheral nerve sheath tumor. Epithelioid sarcoma is the most common soft-tissue tumor of the hand, which generally presents as a painless nodule, with or without ulceration, and can mimic Dupuytren nodules. These soft-tissue sarcomas must be distinguished from benign tumors using a thorough work-up.
      • Chapman T.
      • Athanasian E.
      Malignant tumors of the hand.
      Malignant melanoma is a class of tumors that comprise 1%–3% of malignant tumors. The hand is a particularly rare site for malignant melanoma and is often diagnosed at a late stage because of early clinical detection being overlooked. Early diagnosis is important and demonstrates improved clinical outcomes.
      • Yang Z.
      • Xie L.
      • Huang Y.
      • et al.
      Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
      ,
      • Cho K.
      • Jung S.T.
      Treatment outcomes of advanced stage malignant melanoma in hand and foot after amputation in Korean patients.
      Excisional biopsy with 2-mm clear lateral margins and deep margins that extend to the subcutis layer are recommended for lesions with suspicion of melanoma. The current excision guidelines range from a 5-mm clear margin for melanoma in situ to a minimum of 2-cm clear margins for melanoma greater than 4 mm in thickness. If these margins are not possible to obtain, adjuvant radiotherapy can be considered. Although adjuvant radiotherapy is primarily used for unresectable lesions and for the prevention of recurrence following lymph node resection, there are no current data to suggest improved overall survival in the latter.
      • Perera E.
      • Gnaneswaran N.
      • Jennens R.
      • Sinclair R.
      Malignant melanoma.
      Chemotherapy or biologic therapy alone has shown a poor prognosis for malignant melanoma, and patients often resort to amputation.
      When used as adjuvant therapy following surgical resection or amputation, these therapies demonstrate improved outcomes compared with amputation alone. Yang et al
      • Yang Z.
      • Xie L.
      • Huang Y.
      • et al.
      Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
      reported survival rates of 86.4%, 42.1%, and 31.2% at 1, 3, and 5 years of follow-up, respectively, in patients who received chemotherapy and immunotherapy following digit amputation. Cho et al
      • Cho K.
      • Jung S.T.
      Treatment outcomes of advanced stage malignant melanoma in hand and foot after amputation in Korean patients.
      reported a similar 5-year survival rate in patients who underwent amputation of late-stage malignant melanoma of the hand and foot. These authors reported a poorer prognosis with advanced age, lymph node metastasis, and tumor location on the lower extremities. Thus, they recommended lymph node biopsy to monitor metastasis at follow-up. In our case, the patient was lost to follow-up for 10 months, during which his nodular melanoma tumor had remarkably increased in size. Nodular melanomas, commonly mistaken for hemangiomas or pyogenic granulomas because of their pedunculated appearance, can demonstrate rapid growth over a span of weeks.
      • Perera E.
      • Gnaneswaran N.
      • Jennens R.
      • Sinclair R.
      Malignant melanoma.
      It is also important to note that this patient had a history of trauma to his little finger 4 years prior. There are reported cases of nodular melanoma developing at sites of trauma that may yield the etiology of the tumor in this patient.
      • Jagtap S.V.
      • Khoja S.
      • Billawaria S.
      • Chou T.S.
      • Jagtap S.S.
      Acral nodular melanoma presenting with rapid progression.
      There has been debate regarding local excision versus distal and proximal amputation. Proximal amputation has historically been considered the standard for late-stage tumors with deep tissue invasion; however, more conservative measures have recently been employed to preserve function and cosmetic appearance. Yun et al
      • Yun M.J.
      • Park J.U.
      • Kwon S.T.
      Surgical options for malignant skin tumors of the hand.
      described tissue-preserving treatments, including excision with skin grafts or venous free flaps for malignant melanoma of the hand, but recommend amputation when invasion to the periosteum is suspected. Chakera et al
      • Chakera A.H.
      • Quinn M.J.
      • Lo S.
      • et al.
      Subungual melanoma of the hand.
      reported a similar prognosis with proximal versus distal amputation of subungual melanoma of the hand. Our decision to perform a proximal amputation at the midmetacarpal shaft was primarily because of the patient’s history of noncompliance with recommendations and poor follow-up, which we believed necessitated more aggressive and definitive treatment with a decreased risk of recurrence. Nodular melanoma is characterized by rapid progression and invasive vertical growth patterns through tissue layers, and performing a more proximal amputation was a viable option.
      • Jagtap S.V.
      • Khoja S.
      • Billawaria S.
      • Chou T.S.
      • Jagtap S.S.
      Acral nodular melanoma presenting with rapid progression.
      Further studies should be performed to specifically quantify treatment options for nodular melanoma of the hand.

      References

        • Strike S.A.
        • Puhaindran M.E.
        Tumors of the hand and the wrist.
        JBJS Rev. 2020; 8e0141
        • Chapman T.
        • Athanasian E.
        Malignant tumors of the hand.
        J Am Acad Orthop Surg. 2020; 28: 953-962
        • Turner J.B.
        • Rinker B.
        Melanoma of the hand: current practice and new frontiers.
        Healthcare (Basel). 2014; 2: 125-138
        • Yang Z.
        • Xie L.
        • Huang Y.
        • et al.
        Clinical features of malignant melanoma of the finger and therapeutic efficacies of different treatments.
        Oncol Lett. 2011; 2: 811-815
        • Tomas X.
        • Conill C.
        • Combalia A.
        • Pomes J.
        • Castel T.
        • Nicolau C.
        Malignant melanoma with metastasis into the capitate.
        Eur J Radiol. 2005; 56: 362-364
        • Cho K.
        • Jung S.T.
        Treatment outcomes of advanced stage malignant melanoma in hand and foot after amputation in Korean patients.
        Clin Orthop Surg. 2013; 5: 314-320
        • Perera E.
        • Gnaneswaran N.
        • Jennens R.
        • Sinclair R.
        Malignant melanoma.
        Healthcare (Basel). 2013; 2: 1-19
        • Jagtap S.V.
        • Khoja S.
        • Billawaria S.
        • Chou T.S.
        • Jagtap S.S.
        Acral nodular melanoma presenting with rapid progression.
        J Cutan Aesthet Surg. 2021; 14: 426-432
        • Yun M.J.
        • Park J.U.
        • Kwon S.T.
        Surgical options for malignant skin tumors of the hand.
        Arch Plast Surg. 2013; 40: 238-243
        • Chakera A.H.
        • Quinn M.J.
        • Lo S.
        • et al.
        Subungual melanoma of the hand.
        Ann Surg Oncol. 2019; 26: 1035-1043