Malignant fibrous histiocytoma of soft tissue. An analysis of 78 cases located and deeply seated in the extremities

Seventy-eight cases of malignant fibrous histiocytoma (MFH) of soft tissues are reported. All tumors were deeply located in the extremities. An analysis is presented with regard to sex, age, site, macro- and microscopic features. The staging was assessed according to Enneking's classification (IB, one case; IIA, 25 cases; IIB, 42 cases; III, 10 cases). The recurrence rate after surgical treatment was high (37.5%) with significant differences after inadequate surgery (70%), wide excision (38%), or amputation (0%). Adjunctive radiotherapy rarely was effective in preventing local recurrence. The overall 5-year survival rate was 36%. The prognosis was worse in (1) tumors larger than 5 cm in diameter, (2) tumors that recurred early (less than 1 year) after surgery, and (3) tumors that had inadequate surgical treatment (intralesional or marginal excision).     

Recurrent cutaneous leiomyosarcoma.

BACKGROUND. Soft tissue sarcomas comprise approximately 0.7% of all malignant neoplasms. Superficial leiomyosarcoma, a rare malignant lesion, constitutes 4.0-6.5% of all soft tissue sarcomas, an overall incidence of approximately 0.04% among all cancers. Currently, less than 125 cumulative cases of cutaneous and subcutaneous leiomyosarcoma have been reported in the English literature. METHODS. The authors report the case of a 70-year-old Japanese man with recurrent cutaneous leiomyosarcoma who was treated by wide local excision. A comprehensive literature survey is also presented. RESULTS. The patient is free from recurrence 1 year after wide local excision of a second recurrence of cutaneous leiomyosarcoma. Moreover, superficial leiomyosarcoma can be subdivided into cutaneous leiomyosarcoma and subcutaneous leiomyosarcoma, based on histopathologic and prognostic differences. CONCLUSIONS. The treatment of choice of superficial leiomyosarcoma is wide local excision. Cutaneous leiomyosarcoma is associated with local recurrence only, although subcutaneous leiomyosarcoma undergoes metastatic spread in 30-60% of cases, with a 30-40% mortality rate.     

Histologic features predict local recurrence after breast conserving therapy of phyllodes tumors

Background: Pathologists can distinguish benign phyllodes tumors, which very rarely metastasize, from malignant phyllodes tumors, which metastasize in approximately one fourth of patients. However, whether these same histologic criteria can be used to predict the likelihood that a phyllodes tumor will locally recur after breast conserving therapy remains controversial.Study Design: Since few patients with malignant phyllodes tumors have been treated with breast conserving surgery in any individual series, the literature was reviewed using a Medline search.Results: After local excision, 21 (111/540), 46 (18/39), and 65 (26/40) of patients with benign, borderline, and malignant phyllodes tumors, respectively, recurred in the breast. Following wide local excision, 8 (17/212), 29 (20/68), and 36 (16/45) of patients with benign, borderline, and malignant phyllodes tumors recurred in the breast.Conclusions: Malignant phyllodes tumors are much more likely than benign phyllodes tumors to recur in the breast after breast conserving surgery. This high rate of local recurrence of borderline and malignant phyllodes tumors suggests that wide local excision is less than optimal therapy, and challenges us to look for methods to improve local tumor control.     

Analysis of phyllodes tumor recurrence according to the histologic grade

Local recurrence of phyllodes tumor (PT) of the breast is an adverse outcome that can result in sarcomatous degeneration. The aim of this study was to investigate the histologic and surgical factors associated with local recurrence. A total of 193 PT cases were studied: 145 (75.1 %) benign cases, 33 (17.1 %) borderline cases, and 15 (7.8 %) malignant cases. Stratifying our analysis according to histologic grade, we investigated the relationship between disease-free survival (DFS) and both histologic and surgical factors, including histologic grade, stromal cellularity, stromal atypia, stromal mitosis, stromal overgrowth, tumor margin, type of surgical procedure (local excision, wide excision, and mastectomy), surgical margin status, and radiation therapy. In the case of benign PT, all patients with local recurrences (3.4 %) had been treated with local excision, and all recurrent tumors were also benign. The local recurrence rate for locally excised benign PTs was not associated with surgical margin status or radiation therapy. In the case of borderline PT, local excision was associated with an increased local recurrence rate (P = 0.046). In malignant PT, small tumor size (≤4.0 cm) was associated with an increased local recurrence rate (P = 0.041). Univariate analyses indicated that surgical procedure (mastectomy < local excision < wide excision; P < 0.001) was significantly associated with shorter DFS in borderline PT. A positive surgical resection margin (P < 0.001) was associated with DFS in malignant PT. The factors associated with local recurrence differed with the histologic grade of PT, as did the features of local recurrence itself. In particular, benign PT had very low rate of local recurrence regardless of surgical margin status or radiation therapy, even when treated with local excision. In the case of benign PT, no recurrent tumors had worse histologic grades than the initial tumors.     

Controversies in the surgical management of rectal cancer

At the present time, standard therapy for potentially curable rectal cancer consists of transabdominal surgical resection and adjuvant chemoradiation for American Joint Committee on Cancer stage II/III disease. Controversial issues include the use of local excision as opposed to formal resection and total mesorectal excision (TME) alone without adjuvant therapy. Although early stage tumors are the ideal potential candidates for local excision, clinical staging with endoscopic ultrasound is extremely variable in accurately predicting T and N stage. In addition, even low-grade or T1 tumors are associated with a 7% to 14% chance of nodal metastatic disease. Overall, the risk for local recurrence is higher after local excision but may be reduced by adjuvant therapy. Salvage rates for recurrent disease range from 21% to 91%. In regard to TME, local recurrence rates are an impressive 0% to 12% without adjuvant radiation. However, the addition of radiation therapy may further reduce these already low rates, especially in higher-risk groups. The results of 2 large European studies show acceptable complication rates and the applicability of this technique to a diverse patient population.     

Margins in extra-abdominal desmoid tumors: a comparative analysis

BACKGROUND AND OBJECTIVES: The main treatment of extra-abdominal desmoid tumors remains surgery, but recurrence rates up to 80% are reported. The impact of microscopic surgical margin status according to the Enneking classification system is discussed controversially. METHODS: Therefore, the authors screened the published literature for reliable data on the importance of a wide or radical excision of extra-abdominal desmoid tumors. All studies with more than ten patients, a surgical treatment only, and margin status stated were included. RESULTS: Only 12 out of 49 identified studies fulfilled the inclusion criteria. One hundred fifty-two primary tumors were excised with wide or radical microscopic surgical margins, while in 260 cases a marginal or intralesional excision was performed. In the first group 41 patients (27%) and in the second one 187 patients (72%) developed a recurrence. Therefore, microscopic surgical margin status according to the Enneking classification system is a significant prognostic factor (P < 0.001). CONCLUSIONS: The data of this review underline the strategy of a wide or radical local excision as the treatment of choice. Furthermore, as a large number of studies had to be excluded from this analysis, exact microscopic surgical margin status should be provided in future studies in order to allow comparability. .     

Factors affecting recurrence in lumpectomy without irradiation for breast cancer.

Between 1980 and 1988, 122 women with operable invasive breast cancers underwent wide excision and axillary dissection without subsequent irradiation. During the follow-up period of 1 to 8 years (median, 4 years), recurrences were observed in 23 patients (19%), 22 occurring in the breast and one in the axilla. This is a significant rate of recurrence and supports the need for breast irradiation after conservative surgery. The incidence of recurrence in the breast did not appear to be related to the presence or absence of axillary nodal metastasis. No recurrences were noted in 20 patients whose primary tumors were smaller than 1 cm. The incidence of recurrence was directly correlated to the increasing size of the tumor, but it also appeared to decrease with advancing age. In 31 patients over 70 years of age, only one (3%) recurrence was observed. If these early findings are confirmed, it is likely that patients with tumors smaller than 1 cm or patients over 70 years of age may be spared breast irradiation after wide excision.     

Management of grade III giant cell tumors of bones

BACKGROUND AND OBJECTIVES: Surgical treatment of high-grade giant cell tumors (GCT) of bones with an intralesional excision or a wide excision still poses a dilemma between eradicating the tumor and saving the extremity's function. Our study evaluates the possibility of managing high grade GCT with an intralesional curettage combined with adjuvant therapies, instead of a wide excision, to better avoid limb salvage procedures with endoprostheses. METHODS: According to the grading system of Campanacci et al., twenty-four patients with grade III giant cell bone tumors were treated between May 1983 and Aug 2001 period. We analyzed the differences in local recurrence rates, functional results, and complications between wide excision and curettage with adjuvant therapy in management of the patients diagnosed with primary lesions after a mean follow-up period of 7.5 years (range: 2.1-20.3 years). RESULTS: Fourteen patients underwent the curettage procedure with adjuvant therapy and reconstruction with bone grafts. In the curettage group, two patients had local recurrences and three patients had fracture complications. None of the nine patients receiving wide excisions had experienced local recurrence at the time of their most recent follow-up examination. Using the Musculoskeletal Tumor Society system to evaluate average function, the results were 25.56 points for the wide excision group and 25.64 points for the curettage group, respectively. CONCLUSIONS: Our results suggest that surgical curettage with various adjuvant modalities might be considered as the first choice for treatment of high-grade GCTs in weight-bearing areas, especially in young patients, to avoid unnecessary endoprosthesis.     

Osteosarcoma of the spine: experience of the Cooperative Osteosarcoma Study Group

BACKGROUND: Due to the low incidence rate, the optimal strategy for the treatment of patients with spinal osteosarcoma is unknown. METHODS: Twenty-two patients with osteosarcoma of the spine (15 with tumors of the sacrum and 7 with tumors at other sites) who received chemotherapy according to the Cooperative Osteosarcoma Study Group protocol were analyzed. Six patients presented with metastasis, and 16 patients had no evidence of metastasis at the time of entry into the protocol. Of 12 patients who underwent excision of their tumors, 2 patients underwent wide excision, 3 patients underwent marginal excision, and 7 patients underwent intralesional excision. Eight patients received irradiation: Six patients received conventional radiotherapy only, one patient received neutron beam therapy, and one patient received samarium-153-ethylene diamine tetramethylene phosphonate therapy. Follow-up ranged between 24 months and 105 months (median, 47 months). RESULTS: The median survival was 23 months, and three patients have survived without disease for > 6 years. Patients with primary metastases (P = 0.004), large tumors (P = 0.010), and sacral tumors (P = 0.048) had lower overall survival compared with patients who had no metastasis, small tumors, and nonsacral tumors, respectively. There was a significant difference in overall survival between 5 patients who underwent either wide or marginal surgery and 17 patients who underwent either intralesional surgery or no surgery (P = 0.033). Among 17 patients who underwent no surgery or intralesional surgery, overall survival tended to be better in 7 patients who received irradiation compared with the overall survival in 10 patients who did not receive irradiation (P = 0.059). CONCLUSIONS: Patients with metastases, a large tumors, and sacral tumors had a poor prognosis in the current study with small numbers of patients. Wide or marginal excision of the tumor improved survival. Patients with osteosarcoma of the spine should be treated with a combination of chemotherapy and at least marginal excision for those with surgically accessible tumors. Postoperative radiotherapy may be beneficial.     

Wide excision without radiation for desmoplastic melanoma

BACKGROUND: Adjuvant radiation has been proposed for the treatment of patients with desmoplastic melanoma, who reportedly have local recurrence rates as high as 40-60%. The authors investigated local recurrence rates at a tertiary referral center to determine the success of wide excision alone for patients with desmoplastic melanoma. METHODS: A review of a prospectively maintained melanoma clinical data base identified 65 patients between March 1997 and March 2004 with pure cutaneous desmoplastic melanoma. Complete surgical, histopathologic, and staging information was collected along with data on outcome, including local, regional, and distant recurrence and survival. RESULTS: Similar to previous reports, patients with desmoplastic melanoma had a male-to-female ratio of 2 to 1, a mean age of 65.0 years (range, 31-92 yrs), and the majority of their tumors (55%) were located on the head and neck. The mean Breslow depth at diagnosis was 4.21 mm, with 38% of tumors thicker than 4.0 mm. All patients in this series underwent wide excision without radiation therapy. Surgical margins < or = 2 cm were obtained for all trunk and extremity lesions and for 63% of head and neck lesions that measured > 1 mm in depth (63%). Margins of 1-2 cm were obtained for the remaining patients. Among 49 patients who had a minimum of 2 years of follow-up (mean, 3.7 yrs), the local recurrence rate was 4% (2 of 49 patients). Seventy-eight percent of the patients studied remained alive with no evidence of disease. CONCLUSIONS: Local recurrence rates in the current series were considerably lower than the historically reported rates. This finding suggests that, for patients with desmoplastic melanoma, wide local excision with careful attention to appropriate margins produces excellent local control rates without the need for adjuvant radiation.     

Dermatofibrosarcoma protuberans: treatment and prognosis.

Dermatofibrosarcoma protuberans (DFSP) is a rare tumor of the skin with a strong tendency to recur locally. Nineteen cases of DFSP are presented. In eight of them a total of 20 local recurrences occurred, in five after irradical and in three after 'narrow' excisions. After wide excision (greater than 2 cm) for primary or wide re-excision for recurrent tumor, all patients remained free of tumor with a mean follow-up of 13.2 years (range 2-28 years). An extensive literature review revealed 913 cases of DFSP. The overall recurrence rate is about 50%; after adequate wide excision, 13%. Recurrent tumor is safely treated by wide re-excision and the recurrence rate is then 12%. Regional and distant recurrences are infrequent. Eleven cases (1%) were reported to have regional lymph node metastases and 37 (4%, 17 of whom were histologically confirmed) distant metastases, principally in the lung. The prognosis after appearance of regional or distant recurrence is bad. The role of radiotherapy in the management of this tumor is unclear. Primary or recurrent DFSP is best treated by surgical excision with a minimal margin of 2- preferably 3-cm of surrounding skin including the underlying fascia. Elective lymph node dissection is not advised.     

Salivary gland tumors of the oral cavity

Between 1961 and 1985, 62 patients with malignant salivary gland tumors of the oral cavity underwent surgery with curative intent at the University of California, Los Angeles (UCLA) Medical center. All patients had a minimum follow-up of 2 years. Fifty of 62 (81%) patients presented with T1-2 primary tumors. The tumors arose from the palate in 41/62 (66%) patients. The most common histologic type was adenoid cystic carcinoma comprising 34 of 62 (55%) cases. Radical resection was performed in 46 cases and wide local excision in 16 patients. Postoperative radiation therapy was used in 24 cases due to advanced stage and/or positive surgical margins. Results of treatment were analyzed by stage of disease, modes of treatment, histology, and surgical extent. Local control of small lesions reached 100% at 10 years with either radical resection alone or local excision. With residual tumor at the surgical margins, the incidence of local recurrence was 4/14 (29%) for those who received adjuvant radiation therapy and 5/10 (50%) for those who did not. The vast majority of mucoepidermoid carcinomas were early stage and low grade. These lesions had an excellent prognosis with a control rate of 100%. In contrast, there was a 29% (10/34) failure rate for adenoid cystic carcinoma. The 5-, 10-, and 15-year actuarial survivals for the whole group were 94%, 84%, and 73%, respectively. Our results indicate that for early stage disease, wide local excision may offer patients the chance to avoid the cosmetically and functionally debilitating effects of radical surgery without compromising treatment outcome. Adjuvant radiation therapy appears to reduce the local recurrence for those with residual tumor at the surgical margins.     

Function preserving surgery of limb sarcomas: history and improvements]

In the seventies, limb-sparing surgery and radiation therapy appeared capable of successfully treating the great majority of adult patients with soft tissue sarcomas of the extremities. No survival benefit could be demonstrated in the patients selected for amputation. Microscopically positive surgical margins are related to a greater risk of local recurrence, whereas overall survival is compromised by high grade and large tumor size. Presurgery multidisciplinary discussion, management in reference center and management within cancer network independently predict conformity to the clinical practice guidelines. Reoperation should be a planned part of definitive management whenever the initial surgical procedure was done without a histologic diagnosis or was not planned to be a wide excision. Soft tissue flap reconstruction facilitates therapy for patients with locally advanced tumors so that patients with larger tumors can undergo resection, limiting complications and limb sacrifice. Vascular reconstruction is a feasible option in case of involvement of major vessels. Isolated limb perfusion (ILP) with TNF and chemotherapy is an efficient limb-sparing neoadjuvant therapy for locally advanced limb soft tissue sarcomas. Efficacy and safety of low-dose TNFalpha could greatly facilitate ILP procedures in the near future.     

Intraoperative radiation therapy for patients with recurrent rectal and sigmoid colon cancer in previously irradiated fields.

A retrospective study evaluated 15 patients with pelvic recurrence of colorectal cancer in a previously irradiated region who received intraoperative radiation therapy (IORT) as part of salvage therapy. Total prior external beam radiation therapy (EBRT) doses ranged from 45 to 79.2 Gy. Tumor resection was accomplished in 14 patients, with an exenteration performed in seven. IORT dose was 15-20 Gy. Three patients received additional EBRT as a post-operative course of 25.2 Gy in 14 fractions. Actuarial 3-year local control rate was 25%. The 3-year overall survival rate was 29%. Patients with fixed and/or bulky pelvic tumors had a local control rate of 19% at 12 months and median overall survival of 9 months. Patients with less extensive clinical presentations of anastomotic non-fixed transmural recurrence, isolated pelvic node metastasis and rectal recurrence following local excision had a local control rate of 42% at 36 months and median survival of 43 months. We conclude that clinical presentation of recurrent disease is an important prognostic factor. The value of IORT may be limited to patients with less extensive clinical presentations.     

Large, deep, high-grade extremity sarcomas: treating tumors of the flexor fossae

Limb salvage is now possible for the majority of patients with extremity sarcomas. Although overall prognosis is primarily based on tumor size and histologic grade, complete surgical excision and local control is essential for cure. There are, however, certain anatomic locations such as the flexor fossae in which a complete surgical margin is difficult to attain, and surgery without adjuvant therapy has a high local failure and amputation rate. We have found that preoperative adjuvant therapy consisting of chemotherapy and radiation followed by surgical excision with tumor-free margins has been successful in treating flexor fossa sarcomas with high limb salvage (96%), local control (89%) and overall survival rates (70%). These results are comparable to patients with similar large, high-grade extremity tumors in other compartmental locations.     

Extraskeletal myxoid chondrosarcoma: a Multi-Institutional Study of 42 Cases in Japan

BACKGROUND: Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant neoplasm. Despite a consensus for the distinct clinicopathologic entity of EMC, its clinical features remain controversial. In addition, most studies have contained a small number of patients who underwent definitive surgical treatment. METHODS: Forty-two cases of EMC, which had been identified from files of eight affiliated hospitals and confirmed for histologic diagnosis at the Pathology Center, were analyzed for histologic grade, demographics, treatments, outcomes, and prognostic factors. The average follow-up period was 7.4 years. RESULTS: Included in the study were 20 men and 22 women with a mean age at diagnosis of 52.1 years. The tumors were located mainly in the lower extremities (69%). Thirty-three tumors (79%) were classified as Grade 1 and nine as Grade 2 according to the modified French System. Overall survival was 100% at 5 years and 88% at 10 years. Disease-free survival was 45% at 5 years and 36% at 10 years. Inadequate initial surgery was defined as a significant risk factor for local recurrence by univariate analysis of all 42 patients but not by the analysis of those 30 patients who had undergone wide tumor excision or amputation. Wide excision led to the recurrence rate of 14%. CONCLUSIONS: These findings supported the role of wide excision in the local control of EMC, irrespective of the previous excision procedure or recurrence. The protracted clinical course of the tumors and the presence of patients who had distant metastasis develop after definitive surgery of the primary tumor represented EMC as intermediate malignancy.     

Outcomes and factors impacting local recurrence of ductal carcinoma in situ

BACKGROUND: The optimal management of ductal carcinoma in situ (DCIS) remains controversial. Investigators have focused on identifying patients who are eligible for treatment by excision alone. A retrospective analysis of patients with DCIS treated by various modalities was conducted to compare outcomes and determine factors significant for local recurrence (LR). METHODS: Between 1985-1992, 88 consecutive diagnoses of DCIS were identified in 85 patients. Seventy-four percent were detected mammographically. The most common histologic subtypes were comedo (54%) and cribriform (23%). Tumor sizes were < 2.5 cm (49%), > 2.5-5 cm (26%), > 5 cm (23%), and unknown (2%). Final resection margins were tumor free (75%), close/positive (23%), and unknown (2%). Treatment methods included mastectomy (30%), localized surgery and radiation therapy (LSR) (43%), or wide localized surgery alone (LS) (27%). Radiation therapy (RT) was comprised of 50 grays to the breast, and 53% of treated patients received local "boost" irradiation. RESULTS: The median follow up was 8.3 years. The overall recurrence rate was 13. 6%, whereas the median time to LR was 27.8 months. Recurrence rates according to treatment modality were: LS: 25%; LSR: 13%; and mastectomy: 4%. However, if surgical margins were tumor free, LSR had a LR rate of 3.4%. After RT, no LR occurred prior to 15 months, and 4 of 5 tumors were noninvasive. Nine patients treated by excision alone conformed to the criteria of Lagios et al. criteria and LR occurred in three of nine tumors. Of the factors analyzed, margin status was found to be the best predictor for LR (P = 0.05). CONCLUSIONS: If surgical margins are tumor free, the LSR regimen is equivalent to mastectomy for local tumor control. Annual mammograms may be adequate for the follow-up of patients with irradiated breasts, but biannual studies still are recommended for patients treated with excision alone.     

Optimal management of ductal carcinoma in situ of the breast

Ductal carcinoma in situ (DCIS) represents a breast lesion that is diagnosed with increasing frequency, mainly due to the wide use of screening mammography. Today, DCIS comprises 15–25% of all breast cancers detected at population screening programs. Consequently, the concepts of properly managing such patients assume a greater importance in everyday practice. Mammographically detected microcalcifications are the most common presentation of DCIS. Despite recent technological advances (including Stereotactic-guided directional vacuum-assisted biopsy), mammographically guided wire biopsy remains the “gold-standard” for obtaining a histological diagnosis in patients with non-palpable, mammographically detected DCIS. Management options include mastectomy, local excision combined with radiation therapy, and local excision alone. Given that DCIS is a heterogeneous group of lesions rather than a single entity, and because patients have a wide variety of personal needs that must be addressed during treatment selection, it is obvious that no single approach will be appropriate for all forms of DCIS or for all patients. Careful patient selection is of key importance in order to achieve the best results in the management of the individual patient with DCIS. Axillary lymph node dissection is unnecessary in the treatment of pure DCIS, but it is indicated when microinvasion is present. In these cases, sentinel lymph node biopsy may be an excellent alternative. In the NSABP B-24 trial, tamoxifen reduced both the invasive and non-invasive breast cancer events in either breast by 37%. Nearly all patients who develop a non-invasive recurrence following breast-sparing surgery are cured with mastectomy, and approximately 75% of those with an invasive recurrence are salvaged. Selected patients initially treated by lumpectomy alone may also undergo breast-conservation therapy at the time of relapse according to the same strict guidelines of tumor margin clearance required for the primary lesion; radiation therapy should be given following local excision. The use of systemic therapy in patients with invasive recurrence should be based on standard criteria for invasive breast cancer.     

A randomized clinical trial of adjuvant chemotherapy for radically resected locoregional relapse of breast cancer: IBCSG 27-02, BIG 1-02, and NSABP B-37

In this phase III, multinational, randomized trial, the International Breast Cancer Study Group, Breast International Group, and the National Surgical Adjuvant Breast and Bowel Project will attempt to define the effectiveness of cytotoxic therapy for patients with locoregional recurrence of breast cancer. We will evaluate whether chemotherapy prolongs disease-free survival and, secondarily, whether its use improves overall survival and systemic disease-free survival. Quality of life measurements will be monitored during the first 12 months of the study. Women who have had a previous diagnosis of invasive breast cancer treated by mastectomy or breast-conserving surgery and who have undergone complete surgical excision of all macroscopic disease but who subsequently develop isolated local and/or regional ipsilateral invasive recurrence are eligible. Patients are randomized to observation/no adjuvant chemotherapy or to adjuvant chemotherapy; all suitable patients receive radiation, hormonal, and trastuzumab therapy. Radiation therapy is recommended for patients who have not received previous adjuvant radiation therapy but is required for those with microscopically positive margins. The radiation field must encompass the tumor bed plus a surrounding margin to a dose of >or= 40 Gy. Radiation therapy will be administered before, during, or after chemotherapy. All women with estrogen receptor-positive and/or progesterone receptor-positive recurrence must receive hormonal therapy, with the agent and duration to be determined by the patient's investigator. Adjuvant trastuzumab therapy is permitted for those with HER2- positive tumors, provided that intent to treat is declared before randomization. Although multidrug regimens are preferred, the agents, doses, and use of supportive therapy are at the discretion of the investigator.     

Prognosis of level V malignant melanoma

Level V melanomas have been reported to have a poor prognosis, but in-depth analyses of prognostic factors and treatment have not been reported. From 1952 through 1982, 41 patients presented with primary Clark's Level V melanomas. There were 23 patients who presented with clinical Stage I disease and 18 with Stage II. Among Stage I patients, 9 were treated by wide excision alone and 13 underwent wide excision plus prophylactic regional lymph node dissection (RLND); 8 of 13 patients had histologically positive nodes. Twelve Stage II patients were treated by wide excision and RLND (including three hemipelvectomies), and four refused surgery. The 5-year survival was 52%. For Stage I patients, survival was 62% and disease-free survival (DFS) 28% at 5 years; 6 of 10 recurrences were local or regional only. Prophylactic RLND reduced the incidence of recurrence but did not appear to influence survival rates. Among 14 evaluable Stage II patients, overall survival was 60% and DFS 42% at 3 years; of 4 patients who subsequently had a recurrence, 3 had distant metastases. All seven patients with distant metastases at the time of first recurrence died of disease within 14 months (median, 4 months) of detection of metastatic disease. Primary melanomas of the foot (11 patients) and trunk (4 patients) appeared to have a worse prognosis than other sites. Ulceration (seen in 21 patients) did not appear to significantly influence outcome. These data suggest that most patients with Level V melanoma present with clinically localized disease. Prophylactic RLND did not significantly affect overall survival. The invasiveness of these deep tumors appears to reduce the influence of other factors, including primary site, sex, race, and ulceration. The prognosis of patients with Level V melanoma, even with clinically or histologically positive lymph nodes, is not hopeless, and these patients should be treated aggressively.