Soft Tissue Tumors of the Hand. 2. Malignant

Soft tissue tumors of the hand arise from skin, subcutaneous tissue, tendons, nerve, and blood vessels. Many of these lesions occur on other parts of the body; however, the hand remains a unique site because these tumors have symptoms, appearances, treatments, and prognoses that may be quite different than when on other parts of the body. Their characteristics and the severity of symptoms vary markedly depending on the exact location, size, and type of tumor—and many of these tumors can have multiple forms of presentation. Two articles are intended to provide an overview of benign (previous article) and malignant tumors (this article) of the hand. The rarer and more deleterious tumors are discussed in detail while the common tumors and epidermal lesions with which practitioners are familiar are briefly overviewed. At the completion of these review articles, participants should be able to identify and diagnose various benign and malignant hand tumors as well as understand the accepted current treatment of these growths.     

Benign skin and soft-tissue tumors of the hand

Tumors of the hand are relatively rare when compared to other body regions; the majority are benign in nature. Skin and soft-tissue benign tumors can be classified according to their tissue or origin. Proper diagnosis and differentiation of benign from premalignant or malignant lesions are the most important steps in the management of hand tumors. Surgical excision under adequate anesthesia with histologic diagnosis is the commonest and most accepted treatment.     

Malignant tumors of the hand and wrist

Malignant tumors in the hand and wrist compose a wide variety of lesions involving skin, soft tissues, and bone. Although these lesions are found elsewhere in the body, many have unique characteristics at this anatomic location. Skin tumors predominate; the most common are squamous cell carcinomas, followed in frequency by basal cell carcinomas and malignant melanomas. Other soft-tissue malignancies are less common but may present more difficult diagnostic problems. They often appear as painless masses that sometimes have been present for months or even years and deceptively appear to be benign. A missed or delayed diagnosis of these tumors can have devastating consequences. Bone malignancies involve both primary lesions, of which chondrosarcomas are the most common, and metastatic lesions. Regardless of cell type, treatment of malignant tumors in the hand and wrist requires special considerations because of the important function of these structures. Orthopaedic surgeons should be familiar with the spectrum of these tumors, the work-up necessary to arrive at a precise diagnosis, and the treatment that will achieve the most favorable outcome.     

Benign bony and soft tissue tumors of the hand

It is important to be familiar with the wide variety of benign tumors that may present in everyday hand surgery practice. The treatment of many of these tumors in the hand is based on studies with small numbers of patients or the treatment of similar lesions elsewhere in the body. The purpose of this review is to summarize the recent literature relevant to benign bony and soft tissue tumors in the hand.     

Tumorlike lesions and benign tumors of the hand and wrist

A broad spectrum of tumorlike lesions and neoplasms can occur in the hand and wrist, although with somewhat less frequency than in other parts of the body. A thorough understanding of the differential diagnosis of these lesions and a comprehensive strategy for evaluation are central for effective care. Plain radiographs are diagnostic for most bony lesions, whereas magnetic resonance imaging may be necessary to help differentiate a benign soft-tissue lesion from the rare malignant neoplasm. In spite of the complex anatomy, adherence to proper oncologic principles most often will lead to a satisfactory outcome.     

胰高糖素瘤的诊治

目的回顾性分析国内文献报告的胰高糖素瘤病例,总结其在我国的流行病学资料、临床表现、定性定位诊断及治疗。方法应用中国医学科学院医学信息研究所的“中国生物医学文献数据库”光盘(1981.1-2004.5),检索出20年国内报道的胰高糖素瘤文献,依据纳入标准,得到有效文献28篇,共34例,按肿瘤大小、部位、良恶性、胰外转移、临床表现、影象学检查、治疗方法、术式、转归等项目进行分析。结果34例中,男22例,女12例,平均年龄47.6岁,肿瘤单发33例,多发1例,30例位于胰体尾部,4例位于胰头颈部,平均直径5.23cm,恶性占82.8%,良性占17.2%,23例伴胰外转移,坏死性游走性红斑、糖尿病、口角炎、贫血、消瘦是最常见的症状,血浆氨基酸谱分析有助于诊断,B超、CT等影像学检查阳性率较高,手术是最常用的治疗方法,本组治愈率为56%。结论胰高糖素瘤是罕见疾病,恶性居多,临床表现易与湿疹、糖尿病等混淆,致长期误诊,仔细分析配合理化检查可以定性诊断;提高对本病的认识,作到早期诊断、早期手术,可望提高治愈率,改善预后。     

Fu?tumoren: Diagnostik und Therapie

Despite the compact anatomy with thin soft tissue coverage, diagnosis of both benign and malignant tumors of the foot is often delayed. Diagnostic errors are more common than in other body regions, as neoplasias are rarely considered. Barring a few exceptions the foot is not a typical predilection site for malignant musculoskeletal tumors, although, basically any tumor entity of the musculoskeletal system can affect the foot. Delays in specific diagnostic and therapeutic procedures of these lesions can entail serious consequences for patients as tumor size is a major prognostic factor for recurrence-free survival. In cases of an indistinct persistent swelling or bone lesion a tumorous process should always be considered to ensure early diagnosis and therapy of foot tumors.     

Tumors of the foot: diagnostics and therapy

Despite the compact anatomy with thin soft tissue coverage, diagnosis of both benign and malignant tumors of the foot is often delayed. Diagnostic errors are more common than in other body regions, as neoplasias are rarely considered. Barring a few exceptions the foot is not a typical predilection site for malignant musculoskeletal tumors, although, basically any tumor entity of the musculoskeletal system can affect the foot. Delays in specific diagnostic and therapeutic procedures of these lesions can entail serious consequences for patients as tumor size is a major prognostic factor for recurrence-free survival. In cases of an indistinct persistent swelling or bone lesion a tumorous process should always be considered to ensure early diagnosis and therapy of foot tumors.     

DNA-PCNA dual-staining analysis of bone and soft tissue tumors with flow cytometry

To detect the biological behavior of bone and soft tissue tumors or tumorous lesions is often difficult, but it is essential when planning therapeutic strategy. The DNA-Proliferating-cell nuclear antigen (PCNA) dual-staining analysis method, using flow cytometry, was employed to discover whether it could be of any use in this problem. Specimens were obtained from 9 benign bone lesions, 9 benign soft tissue lesions, 6 malignant bone tumors (3 low-grade, 3 high-grade), and 15 malignant soft tissue tumors (8 low-grade, 7 high-grade). From each of these specimens, DNA histograms, PCNA scores, and cell cycle fractions were acquired and analyzed. There was no significant relationship between DNA ploidy or the PCNA score and the malignancy of the tumors. However, a high PCNA score was observed in the recurrent lesions of four benign tumors. High-grade malignant tumors showed significantly higher PCNA scores than low-grade malignancies (P=0.01). All except one benign lesions showed G0, G1, and M fractions only. In malignant tumors, the G2 cell fraction was significantly higher in high-grade than in low-grade malignancies (P=0.01). One patient with a malignant tumor with marked G1, S, and G2 fractions showed remarkable response to preoperative chemotherapy and radiation therapy. These findings indicate that this method has the ability to disclose the biological nature of cells and provide important information on bone and soft tissue tumors.     

Nd-YAG laser therapy in the field of pulmonary surgery

Since 1980, we have treated 71 patients with malignant tumors and benign lesions by the Nd-YAG laser. The flexible fiberoptic bronchoscope with the local anesthesia was used for the treatments. Fifty-four cases were malignant tumors and 17 were benign lesions involving trachea and/or hilar bronchus. In malignant tumors, 41 cases were lung cancers, 24 of which were squamous cell carcinomas. Of 17 cases of benign lesions, 10 (58.5%) were introgenic stenoses. Palliative treatments were done for 36 cases of lung cancers, of which 23 died within 1 year after treatments. Four of 5 cases, which survived beyond 2 years, were adenoid cystic carcinomas. In 17 benign cases, 13 (76.5%) showed distinct improvements of symptoms and findings after treatments. Three patients died of massive bleedings. In these cases, severe stenoses were observed, which required treatments repeatedly. With the proper knowledge of the indications, limitations and complications, of YAG laser therapy it can be a beneficial mean for the management of benign lesions and malignant tumors in trachea and hilar bronchus.     

Diagnosis and treatment of sacral and retrorectal tumors. II

Forty-three sacral and retrorectal tumors encountered at the Cleveland Clinic were reviewed, with emphasis on incidence, diagnosis, and management. Benign tumors could be differentiated from malignant lesions on the basis of history, physical examination, and radiologic studies. CT scan and Magnetic Resonance Imaging are the most useful tests for staging. Small benign tumors may be removed through a posterior approach. All malignant lesions, and benign lesions greater than 3-4 cm in size should be removed through a combined anterior and posterior approach. All tumors should be completely removed wherever possible, since both benign and malignant tumors will recur when excision is incomplete. Radiotherapy and chemotherapy may provide some palliation for malignant tumors, but these modalities are not curative in our experience.     

Aspiration cytology of soft-tissue tumors. The 10-year experience at an orthopedic oncology center

Since 1972, we have included fine-needle aspiration cytology in the pre-operative evaluation of soft tissue lesions referred to our Orthopedic Oncology Group. In 365 consecutive patients the cytodiagnosis was correctly malignant in 66/74 tumors and correctly benign in 260/271 lesions; cytology was non-diagnostic in four sarcomas and 16 benign lesions. The final pre-operative diagnosis should be based on all pre-operative data to minimize the effect of any misjudgement as regards cytodiagnosis; only two of the 19 false cytodiagnoses were of consequence for the patient. We conclude that aspiration cytology used in this way is a valuable adjunct to determine the further management of soft tissue tumors.     

Primary cardiac tumors.

Cardiac tumors are a rare, but potentially curably form of heart disease. A high index of clinical suspicion is necessary for diagnosis as these tumors have protean manifestations that mimic a variety of other cardiac and noncardiac diseases. Presently, M-mode and two-dimensional echocardiography are utilized as safe, reliable, and noninvasive imaging modalities. Seventy-five per cent of these tumors are benign, with myxoma accounting for 50% and rhabodomyoma comprising 20% of lesions. Various histologic types of sarcoma are the predominant malignant cardiac neoplasms. With strict attention to avoiding perioperative tumor embolization, surgical resection of these lesions can be accomplished with minimal morbidity and mortality. Sixteen consecutive primary tumors of the heart have been surgically treated at Duke University Medical Center since 1966 with no perioperative deaths and no late recurrences.     

Premalignant tumors and conditions of bone.

Most bone sarcomas arise in apparently normal bone. However, some sarcomas arise in preexisting benign bone tumors or in nonneoplastic conditions. Some of the lesions, such as multiple exostoses and Ollier's disease, have a propensity to develop chondrosarcoma. Rarely does a benign giant cell tumor become malignant without prior irradiation, malignant transformation of other benign tumors, such as osteoblastoma and chondroblastoma, is a medical curiosity. Among nonneoplastic conditions, radiation changes, long-standing chronic osteomyelitis, and Paget's disease have definite premalignant connotations. However, the risk of developing cancer in these conditions is still low.     

Tumorous lesions of the hand

A retrospective study was conducted of 543 tumorous lesions of the hand seen in a busy office practice for a 5-year period from April 1976 to April 1981. The lesions were grouped as benign lesions, of either the soft tissue or skeletal type, or as malignant lesions. The overall chance that a hand tumor was malignant was 2% (11 of 543). There was significant association of palmar radial ganglion and carpal tunnel syndrome (15%). Ganglions recur more often after aspiration than after surgery. Certain lesions, particularly a mucous cyst, have a high postoperative complication rate. A few of these unique lesions are described.     

Primary tumors of the small bowel

Primary tumors of the small bowel are uncommon, representing less than 6 per cent of all gastrointestinal tumors and less than 2 per cent of all malignant gastrointestinal tumors. This report concerns a twenty-five year survey of our clinical records from 1946 to 1971 which revealed 140 primary small bowel tumors, excluding periampullary tumors. Fifty-two of the neoplasms (37 per cent) were benign; eighty-eight (63 per cent) were malignant and included twenty-eight adenocarcinomas (31.8 per cent), twenty-four lymphosarcomas (27.3 per cent), nineteen carcinoids (21.6 per cent), and ten leiomyosarcomas (11.4 per cent). The average age at the time of diagnosis was 56.9 years for patients with benign tumors and 55.9 years for those with malignant tumors. The illusive and obscure nature of small bowel tumors is illustrated by the fact that 63.3 per cent of patients with benign lesions and 47.6 per cent of those with malignant lesions had symptoms for more than six months before the diagnosis was made. Bleeding was the most common present complaint in patients with benign neoplasms (52.9 per cent) whereas patients with malignant lesions more often had symptoms of obstruction (50.6 per cent). Most of the benign lesions were located proximally in the small bowel (duodenum, 34.6 per cent; ileum, 11.5 per cent), and most of the malignant lesions were located distally (duodenum, 17.0 per cent; ileum, 61.4 per cent). Treatment of patients with malignant lesions was radical excision whenever possible. Adjunctive radiation therapy was used for those with lymphoma. A second benign or malignant tumor occurred in 42.9 per cent of the patients with primary small bowel tumors. The average period of survival after diagnosis of a malignant small bowel tumor was 5.03 years: for patients with adenocarcinoma, 3.6 years; lymphosarcoma, 1.3 years; carcinoid, 6.8 years; and leiomyosarcoma, 8.3 years.     

Fine-needle aspiration cytology of mucocelelike tumors of the breast.

Mucocelelike tumors of the breast encompass a spectrum of pathologic lesions, including benign tumor, atypical ductal hyperplasia, carcinoma in situ, and colloid carcinoma. Because the fine-needle aspiration (FNA) cytology of mucocelelike tumors covering this pathologic spectrum is not well defined, a study of 21 cases of mucocelelike tumors was conducted. Benign lesions are likely to be poorly cellular and to contain cohesive clusters of cytologically bland cells arranged in two dimensional sheets in the background of abundant mucoid material. Colloid carcinomas are usually highly cellular and contain loosely cohesive clusters and dissociated cells with nuclei showing minimal to mild atypia. The most discriminating feature between benign and malignant lesions appears to be the presence of many dissociated cells with intact cytoplasm. Cases with atypical ductal hyperplasia, with some bordering on carcinoma in situ as seen in 7 of the 12 benign cases, may be difficult to identify on FNA cytology, possibly because of sampling. As expected, some of the atypical cases have intermediate features of benign and malignant tumors. Because of overlapping features in borderline cases, we recommend excisional biopsy for all mucocelelike lesions. Myxoid fibroadenoma is more cellular than benign mucocelelike lesions and can be distinguished from carcinoma by the absence of dissociation and presence of numerous bare nuclei of bland morphology in the background. The mucoid material of myxoid fibroadenoma stained brightly pink rather than magenta as in mucocelelike tumors using the Diff Quik stain.     

Malignant fibrous histiocytoma of the forearm: report of a case and review of the literature.

The tissue histiocyte can give rise to a variety of lesions, either benign or malignant, and the latter often is confused with other sarcomas. The multipotential nature of the histiocyte results in the presence of at least five different cell types in these tumors, and all may be derived from a common precursor cell. The anaplasia of elements which differentiate as fibroblasts appears to correlate with survival. The tumors may be highly malignant, and the 10 year survival rate approximates 40%. Aggressive surgical management of these tumors is mandatory, with either wide en bloc resection or primary amputation of the involved extremity. Should the tumor recur locally after a wide resection and there be no detectable metastases, prompt amputation is indicated.     

Malignant calcifying epithelial odontogenic tumor of the mandible: report of a case with pulmonary metastasis showing remarkable response to platinum derivatives.

We describe a case of CEOT of the mandible, which underwent malignant transformation and developed metastatic tumors of the lung after repeated local recurrence. The primary tumor revealed typical histological features of benign CEOT showing sheets of polyhedral epithelial cells associated with abundant eosinophilic amyloid-like materials. On the other hand, the locally recurrent tumors had malignant features, such as increased nuclear pleomorphism with frequent mitotic figures and vascular invasion of tumor cells, as well as increased proliferative activity assessed by immunostaining for Ki-67. Chemotherapy was carried out against the pulmonary metastatic lesions, which showed a drastic response after 3 courses of intravenous administration of cisplatin (CDDP). To date, a total of 6 courses of CDDP and 6 courses of nedaplatin (CDGP) have been done, and the remaining pulmonary tumors have been dormant. This suggests that platinum derivatives could be a chemotherapeutic agent of choice against this rare tumor.