- Email: [email protected]
Liposarcoma of the Great Toe: A Case Report
The Journal of Foot & Ankle Surgery xxx (2014) 1–4
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Case Reports and Series
Liposarcoma of the Great Toe: A Case Report Jessica Daniels, MRes 1, Carl Green, MBChB, BMedSci (Hons), MRCS 2, Ashok Paul, MBBS, FRCS (Ed), FRCS (Ed), Orth 3 1
Medical Student, University of Manchester, Manchester, United Kingdom Specialist Registrar, Trauma and Orthopaedic Surgery, Manchester Royal Infirmary, United Kingdom 3 Consultant, Trauma and Orthopaedic Surgery, Manchester Royal Infirmary, United Kingdom 2
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Soft tissue sarcomas are rare malignancies, of which liposarcomas are the most common. Pleomorphic liposarcoma accounts for 5% of liposarcoma diagnoses and most commonly presents in the thigh. A 57-yearold female presented with a 5-year history of a persistent, painless swelling of the left great toe that had been previously diagnosed and treated as an in-grown toenail. After magnetic resonance imaging and core biopsy, a grade 2 pleomorphic liposarcoma was diagnosed. Treatment consisted of neoadjuvant radiotherapy and amputation of the great toe and proximal half of the first metatarsal with primary closure. The patient had no evidence of local recurrence or metastatic disease after 1 year of follow-up and, with the use of a prosthesis, had a good functional outcome. This is the first documented presentation of a high-grade pleomorphic liposarcoma of the great toe to our knowledge. Although soft tissue sarcomas are rare, a high index of suspicion is required by clinicians when presented with a soft tissue mass to promptly diagnose and treat these potentially fatal lesions. Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: diagnosis ingrown toenail malignancy neoadjuvant radiotherapy pleomorphic tumor
Soft tissue sarcoma (STS) is a type of malignancy derived from the connective tissue that accounts for approximately 1% of all adult malignancies. Liposarcoma is the most common type of STS, accounting for 20% of all adult STS diagnoses and typically presents in the sixth decade of life (1,2). Three subtypes of liposarcoma have been recognized: well-differentiated/dedifferentiated, myxoid/round cell, and pleomorphic. Pleomorphic liposarcoma (PLS) is the rarest subtype, constituting 5% of all liposarcomas, and is characterized by the presence of lipoblasts and nuclear pleomorphism. Patients will typically present with a slow-growing painless lump deep within the limb musculature or retroperitoneum (1). The 5-year survival of patients diagnosed with liposarcoma has been 82%. The diseasespecific 5-year survival of patients with PLS has been 53% (2), because patients diagnosed with this type of sarcoma will be more likely to develop metastatic disease than those with other types of liposarcoma (3). PLS has rarely been seen within the foot (4–6), with only 2 cases reported in published studies and no known documented cases of PLS affecting the great toe. We present a rare case of a high-grade PLS that presented as a persistent, ingrown toenail of the left great toe.
Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Carl Green, MBChB, BMedSci (Hons), MRCS, Trauma and Orthopaedic Surgery, Manchester Royal Infirmary, Manchester, UK. E-mail address: [email protected] (C. Green).
Case Report A 57-year-old female had first presented to her general practitioner with a slow-growing mass of the left great toe 6 years previously. An ingrown toenail was diagnosed, and the patient underwent surgical treatment. However, the mass continued to grow slowly for the next 4 years, with more rapid growth within the most recent 6 months, prompting referral to an orthopedic surgeon. The physical examination revealed a deformed toenail with an associated painless multilobulated mass that had encapsulated the entire distal phalanx to the left first web space (Fig. 1). Movement was uncompromised at the metatarsophalangeal joint but restricted at the interphalangeal joint. The mass was firm, nonmobile, and nonpulsatile and did not transilluminate. The overlying skin was normal in color, with no evidence of peripheral necrosis. The findings from the systemic examination were normal. Magnetic resonance imaging showed a heterogeneous soft-tissue mass measuring 5.7 4.8 6.8 cm that had not infiltrated the surrounding structures but had caused divergence of the first and second digits (Fig. 2). No cortical bony destruction or marrow edema was noted. Contrast enhancement indicated a sarcomatous change within a lipomatous mass. Therefore, the patient was referred to a specialist soft-tissue sarcoma center for additional management as recommended by the national guidelines (7). Subsequently, a core biopsy obtained by a senior radiologist revealed white lesional tissue consisting of mature adipocytes and pleomorphic lipoblasts. In other areas, spindle cells were seen within a myxoid
1067-2516/$ - see front matter Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2014.02.009
2
J. Daniels et al. / The Journal of Foot & Ankle Surgery xxx (2014) 1–4
Fig. 1. Clinical photographs taken at the initial presentation.
medium that also contained lipoblasts. Elsewhere, spindle cell proliferation was noted but with minimal pleomorphism (Table and Fig. 3). The histologic impression was that of a Trojani grade 2, PLS (8). Thoracic computed tomography showed 2 small soft nodules, 2 and 3 mm, in the left upper lobe. A subsequent computed tomography scan performed 3 months later excluded metastatic disease. The imaging and histologic studies were reviewed by the multidisciplinary team, after which the patient underwent neoadjuvant radiotherapy to a dose of 50 Gy, with the aim of reducing the level of amputation, in accordance with the patient’s wishes. However, a repeat
magnetic resonance imaging scan of the foot showed no reduction in the tumor mass. Surgery was performed approximately 6 weeks after radiotherapy completion. The great toe, metatarsophalangeal joint, and distal one half of the first metatarsal were amputated, and primary closure was achieved. Histologic examination of the resected specimen showed that marginal excision had been achieved; therefore, wider excision was performed 3 weeks later with primary closure. The follow-up examination 1 year after surgery showed no evidence of local recurrence or metastatic disease. The patient was using a first ray orthosis and had had a satisfactory functional outcome (Fig. 4).
Fig. 2. Histologic photographs of core biopsy specimens. (A) Fat cells (arrows) of variable shape and size, many with hyperchromatic nuclei. Some fat cells with multivacuolated cytoplasm are present. A background of atypical spindle cells can also seen (100). (B) A closer view of a malignant lipoblast (400). (C) Population of atypical spindle cells (arrows) (100). (D) One very large atypical cell can be seen adjacent to a blood vessel (400). Views show the overall appearance of a pleomorphic liposarcoma.
J. Daniels et al. / The Journal of Foot & Ankle Surgery xxx (2014) 1–4 Table Trojani/FNCLCC Grading System Parameter Tumor Differentiation Grade 0 d 1 Sarcoma closely resembles normal adult mesenchymal tissue 2 Sarcomas with certain histologic typing 3 Embryonal and undifferentiated sarcomas, sarcomas of doubtful type, synovial sarcomas, osteosarcomas, PNET Score Grade 1, score of 2 to 3
Mitotic Count
Tumor Necrosis
d 0 to 9 mitoses/10 HPF
No necrosis <50% necrosis
10 to 19 mitosis/10 HPF 50% necrosis 20 mitoses/HPF
Grade 2, score of 4 to 5
d
Grade 3, score of 6 to 8
Abbreviations: HPF, high power field; PNET, primitive neuroectodermal tumor. Adapted from Fletcher CDM, Unni KK, Mertens F. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARCPress, Lyon, France, 2002. Based on data from Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 33:37–42, 1984.
Discussion PLS is a rare form of STS that most often has presented within the limb musculature and retroperitoneum (6,9). Gebhard et al (10) examined the prevalence of pleomorphic tumors in different areas of the body and found the following: 36.5% in the lower extremity, 20.5% in the internal trunk, 17.5% in the limb girdles, 16% in the upper extremity, and 9.5% in the thoracoabdominal wall. A total of 75% were deep seated and/or extracompartmental. All variants of liposarcoma will typically present as a slow-growing, painless mass (10). Symptoms will occur because of impingement of adjacent structures by a mass effect or from local invasion (11). The insidious nature of presentation can make patient estimation of the clinical duration unreliable, potentially delaying treatment and, therefore, negatively affecting prognosis. Histologically, it can be difficult to differentiate liposarcoma from a simple lipoma, although liposarcoma will be characterized by the presence of lipoblasts with multiple lipid droplets in their cytoplasm (12). PLSs will show nuclear pleomorphism and a degree of hyperchromicity. The round cell/myxoid and pleomorphic variants have a significant risk of local recurrence and metastasis (6). Locally advanced and systemic disease will usually be incurable. This, when combined with a late diagnosis, can prove fatal (3,5,6). Patients with a suspected STS, especially after an atypical history such as in the case we have presented, must therefore be referred to a soft-tissue sarcoma specialist center on an urgent basis (7). This allows experienced clinicians to perform a triple assessment, including clinical history, imaging studies, and biopsy, to formulate a management plain to optimize patient outcome.
3
The main focus of the treatment of PLS should be early local control to prevent disease progression. This can best be achieved by wide or radical limb-sparing surgery involving an experienced surgeon. Amputation should be avoided where possible to award patients a better quality of life after treatment (13). Radiotherapy, both neoadjuvant or adjuvant, has an important role in the management of STS and can reduce the local recurrence of PLS; however, timing of radiotherapy has remained controversial (2,12). Neoadjuvant, or preoperative, radiotherapy is indicated if the size of the radiation field required for postoperative treatment would be likely to cause significant late morbidity, if the tumor is of borderline operability, and if the tumor is known to be radiosensitive (13). The standard regimen for neoadjuvant radiotherapy is a dose of 50 Gy, in 1.8- to 2-Gy fractions, followed by surgery approximately 6 weeks at the completion of radiotherapy. Additional radiotherapy (10 to 16 Gy) can be given postoperatively if the tumor margins are positive. Adjuvant, or postoperative, radiotherapy is standard for all intermediate- or high-grade tumors at a recommended dose of 60 to 66 Gy in 1.8- to 2-Gy fractions. A 2-phase regimen has been commonly used with a shrinking field, with 50 Gy to the initial larger volume followed by 10 to 16 Gy to a smaller tissue volume (14). It is not always required for low-grade tumors, provided adequate margins have been achieved by surgical excision (14). According to O’Sullivan et al (13), no difference has been found with the use of neoadjuvant or adjuvant radiotherapy with regard to local control, distant control, or survival. Neoadjuvant radiotherapy has been associated with a greater risk of wound complications and the need for secondary wound repair (35% versus 17%). Adjuvant radiotherapy was associated with greater rates of late complications such as fibrosis and peripheral edema (13). PLS has had a low response rate to chemotherapy; hence, this has not been a common treatment (3). To our knowledge, 2 cases of PLS within the foot have been reported, and both resulted in amputation. Nishimoto et al (5) reported a burn patient with a PLS arising from the burn scar on the sole of the foot 25 years after the initial insult. An additional mass was noted in the popliteal fossa, and amputation was indicated. The patient died 3 months after the operation of multiple metastases. Br cic et al (15) described a second case of amputation for PLS of the dorsum of a patient’s right foot. Below-the-knee amputation was performed, and, after follow-up of 6 years, no sign of recurrence or metastasis was found. Amputation has also been indicated to achieve local control in other variants of liposarcoma. Matsuo et al (4) presented a case involving recurrence of a well-differentiated liposarcoma occurring plantar to the fourth metatarsal that was treated by amputation of the fourth metatarsal and the cortices of the third and fifth metatarsals, which achieved local control with 5 years of follow-up. Although not the “first line” treatment, amputation provides patients and clinicians with a “last resort” attempt to control local disease (14). It is indicated when the tumor has bridged multiple compartments, has involved neurovascular structures, or amputation
Fig. 3. Magnetic resonance imaging studies of the tumor. T1 images of the (A) dorsal and (B) plantar aspects of the foot. (C) T2 image showing a heterogeneous mass extending to between the first and second metatarsophalangeal joints (arrows in A and C).
4
J. Daniels et al. / The Journal of Foot & Ankle Surgery xxx (2014) 1–4
Fig. 4. Clinical photographs of the patient’s foot 1 year postoperatively, including the orthosis used within her footwear.
with prosthesis will provide better function than a heavily damaged limb due to radical surgery and radiotherapy. It has also been reported that amputation will result in lower rates of local recurrence and the surgical margins will be more likely to be clear (11). After amputation, patients will face the physical difficulty of losing all or part of a limb and the psychological challenge of acceptance (16). Adjustment can be difficult; however, prosthetics can be used to combat the psychological effect and the motility difficulty if a lower limb has been amputated (10). The present patient did not have the option of limb salvage surgery owing to the site of the lesion; however, despite these challenges, she had a good functional outcome. The prognosis after the diagnosis of PLS will be determined by several factors and will be improved by early detection and prompt and appropriate treatment (11). Although the 5-year disease-specific survival for PLS has been 53%, the potential exists to increase this rate with the introduction of biomarker-targeted agents in the future (3). Recurrence has been documented for up to 10 years after the initial resection, although recurrence has most commonly been seen approximately 3 years after surgery (6). This highlights the need for long-term surveillance of these patients after primary surgery and adjuvant treatment. In conclusion, an unusual case of PLS that presented as an ingrown toenail has been reported. The clinical suspicion in this circumstance was initially low, leading to a delayed diagnosis. Our case report will help to demonstrate that rare diagnoses should feature on the list of differential diagnoses to reduce the possible delay in diagnosis and allow prompt investigation and treatment of any potentially fatal lesion. References 1. Wente M, Schwarzbach M, Hinz U, Leowaldi C, Mechterscheimer G, Krempien R, € chler MW. Perioperative outcome in sarcoma surgery. Egerer G, Friess H, Bu Langenbecks Arch Surg 392:83–93, 2007. € fvenberg R, 2. Engstrom K, Bergh P, Gustafson P, Hultborn R, Johansson H, L o € m O, Bauer HC. Liposarcoma outcome based Zaikova O, Trovik C, Wahlstr o
3.
4. 5. 6.
7.
8.
9.
10.
11. 12. 13.
14.
15. 16.
on the Scandinavian Sarcoma Group Register. Cancer 113:1649–1656, 2008. Ghadimi M, Liu P, Peng T, Bolshakov S, Young ED, Torres KE, Colombo C, Hoffman A, Broccoli D, Hornick JL, Lazar AJ, Pisters P, Pollock RE, Lev D. Pleomorphic liposarcoma: clinical observations and molecular variables. Cancer 117:5359–5369, 2011. Matsuo T, Sugita T, Shimose S, Kubo T. Liposarcoma arising in the foot: a case report. Case Rep Med 2009:630203, 2009. Nishimoto S, Matsushita T, Matsumoto K, Adachi S. A rare case of a burn scar malignancy. Burns 22:497–499, 1996. Issakov J, Soyfer V, Kollender Y, Bickels J, Meller I, Merimsky O. Liposarcoma in adult limbs treated by limb sparing surgery and radiotherapy. J Bone Joint Surg Br 88:1647–1651, 2006. National Institute for Health and Clinical Excellence. Guidance on Cancer Services: Improving Outcomes for People with Sarcoma, National Institute for Health and Clinical Excellence. Available at: http://www.nice.org.uk/nicemedia/pdf/ sarcomafullguidance.pdf. Accessed October 25, 2013. Fletcher CDM, Sundaram M, Rydholm A, Coindre JM, Singer S. Soft Tissue Tumors: Epidemiology, Clinical Features, Histopathological Typing and Grading, WHO Classification of Soft Tissue Tumours. Available at: http://www.iarc.fr/en/publications/ pdfs-online/pat-gen/bb5/bb5-classifsofttissue.pdf. Accessed October 25, 2013. € lke E, Zurakowski D, Orth K, Heinecke A, Knoefel WT. Peiper M, Matthaei H, Bo Compartmental resection for subfascial extremity soft tissue sarcoma and quality of life in long-term survivors. Eur J Med 123:488–495, 2011. Gebhard S, Coindre J, Michels J, Terrier P, Bertrand G, Trassard M, Taylor S, Chateau MC, Marques B, Picot V, Guillou L. Pleomorphic liposarcoma: clinicopathologic and immunohistochemical study of 63 cases. A study of the French Sarcoma Group. Am J Surg Pathol 26:601–616, 2002. Beckingsale T, Gerrand C. The management of soft-tissue sarcomas. Orthop Trauma 23:240–247, 2009. Enzinger F, Weiss S. Soft Tissue Tumours, ed 2, Mosby, St Louis, MO, 1994. O’Sullivan B, Davis A, Turcotte R, Bell R, Catton C, Chabot P, Wunder J, Kandel R, Goddard K, Sadura A, Pater J, Zee B. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised control trial. Lancet 359:2235–2241, 2002. Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma 2010:506182, 2010. Available at: http://www.hindawi. com/journals/sarcoma/2010/506182/. Accessed October 14, 2013. Br ci c L, Jakov cevi c A, Vuleti c L, Orli c D, Seiwerth S. Pleomorphic liposarcoma of the foot: a case report. Diagn Pathol 3:15, 2008. Jensen M, Moore M, Bockow T, Ehde D, Engle J. Psychosocial factors and adjustment to chronic pain in persons with physical disabilities: a systematic review. Arch Phys Med Rehab 92:146–160, 2011.
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Case Reports and Series
Liposarcoma of the Great Toe: A Case Report Jessica Daniels, MRes 1, Carl Green, MBChB, BMedSci (Hons), MRCS 2, Ashok Paul, MBBS, FRCS (Ed), FRCS (Ed), Orth 3 1
Medical Student, University of Manchester, Manchester, United Kingdom Specialist Registrar, Trauma and Orthopaedic Surgery, Manchester Royal Infirmary, United Kingdom 3 Consultant, Trauma and Orthopaedic Surgery, Manchester Royal Infirmary, United Kingdom 2
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Soft tissue sarcomas are rare malignancies, of which liposarcomas are the most common. Pleomorphic liposarcoma accounts for 5% of liposarcoma diagnoses and most commonly presents in the thigh. A 57-yearold female presented with a 5-year history of a persistent, painless swelling of the left great toe that had been previously diagnosed and treated as an in-grown toenail. After magnetic resonance imaging and core biopsy, a grade 2 pleomorphic liposarcoma was diagnosed. Treatment consisted of neoadjuvant radiotherapy and amputation of the great toe and proximal half of the first metatarsal with primary closure. The patient had no evidence of local recurrence or metastatic disease after 1 year of follow-up and, with the use of a prosthesis, had a good functional outcome. This is the first documented presentation of a high-grade pleomorphic liposarcoma of the great toe to our knowledge. Although soft tissue sarcomas are rare, a high index of suspicion is required by clinicians when presented with a soft tissue mass to promptly diagnose and treat these potentially fatal lesions. Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: diagnosis ingrown toenail malignancy neoadjuvant radiotherapy pleomorphic tumor
Soft tissue sarcoma (STS) is a type of malignancy derived from the connective tissue that accounts for approximately 1% of all adult malignancies. Liposarcoma is the most common type of STS, accounting for 20% of all adult STS diagnoses and typically presents in the sixth decade of life (1,2). Three subtypes of liposarcoma have been recognized: well-differentiated/dedifferentiated, myxoid/round cell, and pleomorphic. Pleomorphic liposarcoma (PLS) is the rarest subtype, constituting 5% of all liposarcomas, and is characterized by the presence of lipoblasts and nuclear pleomorphism. Patients will typically present with a slow-growing painless lump deep within the limb musculature or retroperitoneum (1). The 5-year survival of patients diagnosed with liposarcoma has been 82%. The diseasespecific 5-year survival of patients with PLS has been 53% (2), because patients diagnosed with this type of sarcoma will be more likely to develop metastatic disease than those with other types of liposarcoma (3). PLS has rarely been seen within the foot (4–6), with only 2 cases reported in published studies and no known documented cases of PLS affecting the great toe. We present a rare case of a high-grade PLS that presented as a persistent, ingrown toenail of the left great toe.
Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: Carl Green, MBChB, BMedSci (Hons), MRCS, Trauma and Orthopaedic Surgery, Manchester Royal Infirmary, Manchester, UK. E-mail address: [email protected] (C. Green).
Case Report A 57-year-old female had first presented to her general practitioner with a slow-growing mass of the left great toe 6 years previously. An ingrown toenail was diagnosed, and the patient underwent surgical treatment. However, the mass continued to grow slowly for the next 4 years, with more rapid growth within the most recent 6 months, prompting referral to an orthopedic surgeon. The physical examination revealed a deformed toenail with an associated painless multilobulated mass that had encapsulated the entire distal phalanx to the left first web space (Fig. 1). Movement was uncompromised at the metatarsophalangeal joint but restricted at the interphalangeal joint. The mass was firm, nonmobile, and nonpulsatile and did not transilluminate. The overlying skin was normal in color, with no evidence of peripheral necrosis. The findings from the systemic examination were normal. Magnetic resonance imaging showed a heterogeneous soft-tissue mass measuring 5.7 4.8 6.8 cm that had not infiltrated the surrounding structures but had caused divergence of the first and second digits (Fig. 2). No cortical bony destruction or marrow edema was noted. Contrast enhancement indicated a sarcomatous change within a lipomatous mass. Therefore, the patient was referred to a specialist soft-tissue sarcoma center for additional management as recommended by the national guidelines (7). Subsequently, a core biopsy obtained by a senior radiologist revealed white lesional tissue consisting of mature adipocytes and pleomorphic lipoblasts. In other areas, spindle cells were seen within a myxoid
1067-2516/$ - see front matter Ó 2014 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2014.02.009
2
J. Daniels et al. / The Journal of Foot & Ankle Surgery xxx (2014) 1–4
Fig. 1. Clinical photographs taken at the initial presentation.
medium that also contained lipoblasts. Elsewhere, spindle cell proliferation was noted but with minimal pleomorphism (Table and Fig. 3). The histologic impression was that of a Trojani grade 2, PLS (8). Thoracic computed tomography showed 2 small soft nodules, 2 and 3 mm, in the left upper lobe. A subsequent computed tomography scan performed 3 months later excluded metastatic disease. The imaging and histologic studies were reviewed by the multidisciplinary team, after which the patient underwent neoadjuvant radiotherapy to a dose of 50 Gy, with the aim of reducing the level of amputation, in accordance with the patient’s wishes. However, a repeat
magnetic resonance imaging scan of the foot showed no reduction in the tumor mass. Surgery was performed approximately 6 weeks after radiotherapy completion. The great toe, metatarsophalangeal joint, and distal one half of the first metatarsal were amputated, and primary closure was achieved. Histologic examination of the resected specimen showed that marginal excision had been achieved; therefore, wider excision was performed 3 weeks later with primary closure. The follow-up examination 1 year after surgery showed no evidence of local recurrence or metastatic disease. The patient was using a first ray orthosis and had had a satisfactory functional outcome (Fig. 4).
Fig. 2. Histologic photographs of core biopsy specimens. (A) Fat cells (arrows) of variable shape and size, many with hyperchromatic nuclei. Some fat cells with multivacuolated cytoplasm are present. A background of atypical spindle cells can also seen (100). (B) A closer view of a malignant lipoblast (400). (C) Population of atypical spindle cells (arrows) (100). (D) One very large atypical cell can be seen adjacent to a blood vessel (400). Views show the overall appearance of a pleomorphic liposarcoma.
J. Daniels et al. / The Journal of Foot & Ankle Surgery xxx (2014) 1–4 Table Trojani/FNCLCC Grading System Parameter Tumor Differentiation Grade 0 d 1 Sarcoma closely resembles normal adult mesenchymal tissue 2 Sarcomas with certain histologic typing 3 Embryonal and undifferentiated sarcomas, sarcomas of doubtful type, synovial sarcomas, osteosarcomas, PNET Score Grade 1, score of 2 to 3
Mitotic Count
Tumor Necrosis
d 0 to 9 mitoses/10 HPF
No necrosis <50% necrosis
10 to 19 mitosis/10 HPF 50% necrosis 20 mitoses/HPF
Grade 2, score of 4 to 5
d
Grade 3, score of 6 to 8
Abbreviations: HPF, high power field; PNET, primitive neuroectodermal tumor. Adapted from Fletcher CDM, Unni KK, Mertens F. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARCPress, Lyon, France, 2002. Based on data from Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 33:37–42, 1984.
Discussion PLS is a rare form of STS that most often has presented within the limb musculature and retroperitoneum (6,9). Gebhard et al (10) examined the prevalence of pleomorphic tumors in different areas of the body and found the following: 36.5% in the lower extremity, 20.5% in the internal trunk, 17.5% in the limb girdles, 16% in the upper extremity, and 9.5% in the thoracoabdominal wall. A total of 75% were deep seated and/or extracompartmental. All variants of liposarcoma will typically present as a slow-growing, painless mass (10). Symptoms will occur because of impingement of adjacent structures by a mass effect or from local invasion (11). The insidious nature of presentation can make patient estimation of the clinical duration unreliable, potentially delaying treatment and, therefore, negatively affecting prognosis. Histologically, it can be difficult to differentiate liposarcoma from a simple lipoma, although liposarcoma will be characterized by the presence of lipoblasts with multiple lipid droplets in their cytoplasm (12). PLSs will show nuclear pleomorphism and a degree of hyperchromicity. The round cell/myxoid and pleomorphic variants have a significant risk of local recurrence and metastasis (6). Locally advanced and systemic disease will usually be incurable. This, when combined with a late diagnosis, can prove fatal (3,5,6). Patients with a suspected STS, especially after an atypical history such as in the case we have presented, must therefore be referred to a soft-tissue sarcoma specialist center on an urgent basis (7). This allows experienced clinicians to perform a triple assessment, including clinical history, imaging studies, and biopsy, to formulate a management plain to optimize patient outcome.
3
The main focus of the treatment of PLS should be early local control to prevent disease progression. This can best be achieved by wide or radical limb-sparing surgery involving an experienced surgeon. Amputation should be avoided where possible to award patients a better quality of life after treatment (13). Radiotherapy, both neoadjuvant or adjuvant, has an important role in the management of STS and can reduce the local recurrence of PLS; however, timing of radiotherapy has remained controversial (2,12). Neoadjuvant, or preoperative, radiotherapy is indicated if the size of the radiation field required for postoperative treatment would be likely to cause significant late morbidity, if the tumor is of borderline operability, and if the tumor is known to be radiosensitive (13). The standard regimen for neoadjuvant radiotherapy is a dose of 50 Gy, in 1.8- to 2-Gy fractions, followed by surgery approximately 6 weeks at the completion of radiotherapy. Additional radiotherapy (10 to 16 Gy) can be given postoperatively if the tumor margins are positive. Adjuvant, or postoperative, radiotherapy is standard for all intermediate- or high-grade tumors at a recommended dose of 60 to 66 Gy in 1.8- to 2-Gy fractions. A 2-phase regimen has been commonly used with a shrinking field, with 50 Gy to the initial larger volume followed by 10 to 16 Gy to a smaller tissue volume (14). It is not always required for low-grade tumors, provided adequate margins have been achieved by surgical excision (14). According to O’Sullivan et al (13), no difference has been found with the use of neoadjuvant or adjuvant radiotherapy with regard to local control, distant control, or survival. Neoadjuvant radiotherapy has been associated with a greater risk of wound complications and the need for secondary wound repair (35% versus 17%). Adjuvant radiotherapy was associated with greater rates of late complications such as fibrosis and peripheral edema (13). PLS has had a low response rate to chemotherapy; hence, this has not been a common treatment (3). To our knowledge, 2 cases of PLS within the foot have been reported, and both resulted in amputation. Nishimoto et al (5) reported a burn patient with a PLS arising from the burn scar on the sole of the foot 25 years after the initial insult. An additional mass was noted in the popliteal fossa, and amputation was indicated. The patient died 3 months after the operation of multiple metastases. Br cic et al (15) described a second case of amputation for PLS of the dorsum of a patient’s right foot. Below-the-knee amputation was performed, and, after follow-up of 6 years, no sign of recurrence or metastasis was found. Amputation has also been indicated to achieve local control in other variants of liposarcoma. Matsuo et al (4) presented a case involving recurrence of a well-differentiated liposarcoma occurring plantar to the fourth metatarsal that was treated by amputation of the fourth metatarsal and the cortices of the third and fifth metatarsals, which achieved local control with 5 years of follow-up. Although not the “first line” treatment, amputation provides patients and clinicians with a “last resort” attempt to control local disease (14). It is indicated when the tumor has bridged multiple compartments, has involved neurovascular structures, or amputation
Fig. 3. Magnetic resonance imaging studies of the tumor. T1 images of the (A) dorsal and (B) plantar aspects of the foot. (C) T2 image showing a heterogeneous mass extending to between the first and second metatarsophalangeal joints (arrows in A and C).
4
J. Daniels et al. / The Journal of Foot & Ankle Surgery xxx (2014) 1–4
Fig. 4. Clinical photographs of the patient’s foot 1 year postoperatively, including the orthosis used within her footwear.
with prosthesis will provide better function than a heavily damaged limb due to radical surgery and radiotherapy. It has also been reported that amputation will result in lower rates of local recurrence and the surgical margins will be more likely to be clear (11). After amputation, patients will face the physical difficulty of losing all or part of a limb and the psychological challenge of acceptance (16). Adjustment can be difficult; however, prosthetics can be used to combat the psychological effect and the motility difficulty if a lower limb has been amputated (10). The present patient did not have the option of limb salvage surgery owing to the site of the lesion; however, despite these challenges, she had a good functional outcome. The prognosis after the diagnosis of PLS will be determined by several factors and will be improved by early detection and prompt and appropriate treatment (11). Although the 5-year disease-specific survival for PLS has been 53%, the potential exists to increase this rate with the introduction of biomarker-targeted agents in the future (3). Recurrence has been documented for up to 10 years after the initial resection, although recurrence has most commonly been seen approximately 3 years after surgery (6). This highlights the need for long-term surveillance of these patients after primary surgery and adjuvant treatment. In conclusion, an unusual case of PLS that presented as an ingrown toenail has been reported. The clinical suspicion in this circumstance was initially low, leading to a delayed diagnosis. Our case report will help to demonstrate that rare diagnoses should feature on the list of differential diagnoses to reduce the possible delay in diagnosis and allow prompt investigation and treatment of any potentially fatal lesion. References 1. Wente M, Schwarzbach M, Hinz U, Leowaldi C, Mechterscheimer G, Krempien R, € chler MW. Perioperative outcome in sarcoma surgery. Egerer G, Friess H, Bu Langenbecks Arch Surg 392:83–93, 2007. € fvenberg R, 2. Engstrom K, Bergh P, Gustafson P, Hultborn R, Johansson H, L o € m O, Bauer HC. Liposarcoma outcome based Zaikova O, Trovik C, Wahlstr o
3.
4. 5. 6.
7.
8.
9.
10.
11. 12. 13.
14.
15. 16.
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