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17 Tumors of the Soft Tissue of the Lower Extremity 17 Tumors of the Soft Tissue of the Lower Extremity Bradley W. Bakotic, D.P.M., D.O. INTRODUCTION arise more commonly in locations that are richer in smooth muscle, such as the uterine corpus. There is some confusion within segments of the medical Tumors of the soft tissue may be classified into three community as to the precise meaning of “soft tissue.” The major categories: nonneoplastic (reactive), neoplastic but soft tissue of the human body includes all extraskeletal tis- benign, and neoplastic and biologically malignant. The sue that is neither epithelial, hematopoietic (marrow derived same may be said of epithelial tumors and those of the skel- blood elements), nor parenchymal (constituent of a visceral etal system. Those tumors that are not neoplastic are com- organ). The nervous system is divided such that neither the posed of a heterogeneous (polyclonal) population of cells. glial nor the central neuronal elements are considered to be In most instances these lesions arise as the result of a stimu- soft tissue, though by convention the peripheral nervous sys- lus and regress when that stimulus is removed. In contrast, tem is. In sum, the soft tissues consist of adipose tissue, neoplasms emanate from a single “mother” cell, and as a fibrous tissue, musculature, vascular structures, and periph- result each cell is genetically identical. Neoplasms do not eral nerves. necessarily arise as the direct result of an external stimulus, It should not be surprising that tumors of the soft tissue but rather begin as the result of a cascade of genetic muta- may arise virtually anywhere in the human body. Although tions that result in cell immortality and uncontrolled prolif- soft tissue predominates in some regions of the human eration. To be certain, some neoplasms evolve as the direct body such as the thigh and retroperitoneum, it is also result of a stimulus (examples: HPV-related carcinoma, found throughout the remainder of the human body in the radiation-related sarcoma); however, such neoplasms do not form of vessels and peripheral nerves. Possibly as a direct regress when such stimuli are removed. result of their extensive distribution, soft tissue tumors that As mentioned, neoplasms may be benign or malignant. are related to those elements may arise in a wide range of As a general rule, benign neoplasms do not become widely locations. For instance, leiomyomas (tumors of smooth invasive and lack the ability to metastasize or cause death. muscle differentiation) may arise in the skin (related to Again, these rules have their limitations. Some benign neo- vessels of the superficial or deep plexi or erector pili mus- plasms such as uterine leiomyomas may seed the lungs. culature), within parenchymal organs, or within the lumina Fibromatoses may be diffusely infiltrative, and those that of vessels themselves. As might be expected, leiomyomas arise within the abdomen may even cause death. By con- vention, malignant neoplasms are those that have metastatic 17-1 Principles and Practice of Podiatric Medicine potential and may cause death. Limitations exist with regard inspection alone. Such masses typically require histopatho- to malignant neoplasms as well. For instance, basal cell car- logic analysis to be definitively identified. Parenthetically, it cinoma is quite innocuous in most cases, and both may be impossible to distinguish soft tissue tumors from metastases and deaths related to this neoplasm are publish- true cysts, granulomatous infiltrates (gout, granuloma annu- able events. lare, and rheumatoid nodule), and tumors of the cutaneous The classification of many soft tissue tumors is in flux as adnexae (see chapter 16) based strictly on their clinical the result of ongoing research, particularly within genetic appearance. arenas. Kaposi’s sarcoma and fibromatosis are examples of Of greatest concern when managing patients with soft tumors whose precise characterization has proven contro- tissue tumors is the potential for confusion between those versial. Kaposi’s sarcoma was originally classified as a sar- soft tissue tumors that are malignant and similarly appear- coma because in extreme cases it may involve the lungs and ing masses that are wholly benign. This concern has been other viscera so extensively as to cause death; however, it is documented on several occasions in relation to synovial sar- now thought by some investigators to be a form of virally coma and its tendency to mimic ganglion cysts when arising induced hyperplasia. Alternatively, superficial and deep on the dorsum of the foot.1,2,5 A feature that might augment fibromatoses (desmoid) have been traditionally described as the clinician’s ability to assess subcutaneous masses of the benign, though these tumors may be locally destructive and, distal lower extremity is tumor localization. Certain soft tis- in the case of desmoid, may lead to death when arising near sue tumors have a predilection for particular sites in the vital structures. This potentially aggressive behavior has led foot. Soft tissue tumors that are most likely to affect the some to suggest that they would be better classified as low- foot’s dorsal surface are ganglion cyst, giant cell tumor of grade malignancies. tendon sheath, Kaposi’s sarcoma, and synovial sarcoma. The trunk and proximal extremities play host to the over- Epidermal “inclusion” cysts and periosteal chondromas are whelming majority of all soft tissue neoplasms; however, also commonly seen in this location.1,2 The most likely soft tissue tumors exhibiting virtually every type of differen- tumors to arise within the pedal digits are ganglion cysts, tiation may also arise within the foot.1,2 Some tumors such epidermal inclusion cysts, giant cell tumor of tendon sheath, as Kaposi’s sarcoma, synovial sarcoma, and clear cell sar- and Kaposi’s sarcoma.1–3 In the Memorial Sloan-Kettering coma are seen in the distal extremity at a disproportionally Cancer Center series, the digital region also played host to high rate relative to more proximal sites.1 In this author’s epithelioid sarcoma in a significant number of cases.1 The series of 401 soft tissue neoplasms of the foot, which was plantar surface of the foot is affected most by superficial assembled at Memorial Sloan-Kettering Cancer Center, the fibromatosis, followed by Kaposi’s sarcoma and epidermal most commonly diagnosed lesions were Kaposi’s sarcoma, inclusion cysts.1,2 The heel is affected by the aforemen- synovial sarcoma, superficial fibromatosis, giant cell tumor tioned tumors at a slightly reduced rate. The region of the of tendon sheath, and clear-cell sarcoma (in descending heel is of particular interest because it is among the most order).1 This study exhibited a pronounced bias toward common sites for clear-cell sarcoma to manifest.1 Clear cell those tumors that appeared clinically malignant, as is com- sarcoma is a highly malignant neoplasm that is also known monly the case with series of the lower extremity that are as melanoma of soft parts.1 assembled at facilities that specialize in the treatment of Soft tissue tumors may pose problems with respect to cancer. In addition, this series was largely limited to neo- both their histopathologic classification and clinical man- plasms, though the author acknowledged that the neoplastic agement. There is a wide spectrum of clinical behavior that nature of both Kaposi’s sarcoma and superficial fibromato- may be attributed to various tumors of the soft tissue. These sis is in debate. When nonneoplastic (reactive) tumors of tumors may range from overtly benign to highly malignant the lower extremity, such as ganglion cysts are included in in nature. Some soft tissue neoplasms may be extremely dif- reviews of the subject, such lesions typically outnumber ficult to classify in terms of their malignant potential based bona fide neoplasms.2,3 on histopathologic features. In many such cases, even the distinction between “benign” and “malignant” may not be entirely clear. It is now accepted that when attempting to Clinical Features characterize the biologic potential of some soft tissue neo- Soft tissue tumors are distinct in that unlike the vast plasms, all is not black or white, but rather, there are often majority of cutaneous carcinomas, most present as non- shades of gray. descript subcutaneous nodules which lack a distinctive clin- To convey more accurately the biologic behavior of sarco- ical appearance. Where most basal cell carcinomas may be mas (malignant soft tissue neoplasms) to treating clinicians, readily identified based strictly on their clinical appearance, the concept of tumor grade was introduced. This schema of soft tissue neoplasms and nonneoplastic soft tissue prolifer- nomenclature allows tumors to be stratified into those that ations cannot be accurately characterized based on clinical may be expected to metastasize quickly and those that are 17-2 Tumors of the Soft Tissue of the Lower Extremity Chapter 17 more likely to remain localized. Neoplasms are graded as 5- and 10-year survival that is comparable to protocols that either low-grade or high-grade, depending on various histo- include more aggressive surgery.6,8 In some centers postop- pathologic criteria such as the degree of cytologic atypia, the erative radiotherapy is reserved for high-grade sarcomas and mitotic rate, and presence or absence of necrosis. This is done applied only to low-grade sarcomas that measure greater in a manner similar to that which is applied to malignant neo- than 5 cm in maximum dimension.7 plasms within the skeletal system. As a general rule, low- grade malignant neoplasms are slow-growing and have a low probability of metastasis. Dermatofibrosarcoma protuberans (DFSP) is an example of a tumor whose malignant status is BIOPSY TECHNIQUES accepted but, due to its bland histopathologic features and limited metastatic potential, is further qualified by the term Most soft tissue tumors lack clinical features that allow low-grade. In contrast, high-grade malignant neoplasms grow them to be distinguished from each other or from other rapidly and readily metastasize.
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