Treatment of malignant fibrous histiocytoma and atypical fibrous xanthomas with micrographic surgery

Fibrous tumors of the soft tissue are usually benign, but some fibrous neoplasms such as dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and malignant fibrohistiocytoma (MFH) can be very destructive locally with a high recurrence rate after local excision. On occasion, they can metastasize. Previous reports have confirmed the high success rate of Mohs micrographic surgery for the treatment of DFSP, but data have been lacking on the potential benefit of this surgical approach for MFH and AFX tumors. Over the past 6 years, we have treated 17 patients with MFH (20 tumors) and 5 patients with AFX with Mohs micrographic surgery. A retrospective analysis of the surgical results is presented. To date (average 3-year follow-up), all patients contacted are tumor free with only one recurrence; no patient has developed metastatic disease. Our results to date are very encouraging; they lend support to Mohs micrographic surgery as a desired surgical approach for these difficult-to-cure neoplasms.     

Dermatofibrosarcoma protuberans: treatment results of 35 cases.

BACKGROUND AND PURPOSE: This study evaluates the treatment results of patients with dermatofibrosarcoma protuberans. PATIENTS AND METHODS: Between August 1987 and July 1998, 35 consecutive patients with pathologically proved dermatofibrosarcoma protuberans received surgery with or without radiation therapy. Their treatment results were analyzed retrospectively. RESULTS: The patient ages ranged from 5 to 67 years (median 37 years). There were 24 males and 11 females. The anatomic sites of tumor were: trunk in 21, extremity in eight, and head and neck region in six. The maximal dimension of tumor ranged from 1.5 to 25 cm. Surgery techniques included local excision and wide excision with or without graft or flap. Adjuvant radiation therapy was given to 11 patients, with a dose ranging from 46 to 68 Gy (one pre-operative, ten post-operative). At a median follow-up of 50 months (range 11-131 months), there were 11 patients (nine patients without radiation therapy) who developed local failure. Salvage therapy (excision with or without radiation therapy) was given to all of them, and ten achieved disease control. Some patients had treatment-related moderate cosmetic or functional problems. CONCLUSIONS: Dermatofibrosarcoma protuberans is a malignancy of a high cure rate, and adjuvant radiation therapy can reliably decrease the local recurrence rate and prevent mutilation and functional deficit caused by repeated surgery.     

Surgical treatment of dermatofibrosarcoma protuberans. A retrospective study of 20 cases with review of literature

The clinicopathological features and results of surgical treatment of 20 patients with dermatofibrosarcoma protuberans (DFSP) were reviewed. All patients have been observed until the present time or death. The primary treatment usually consisted of marginal, wide or radical excision. Re-excision because of recurrence was performed in six of the 20 patients who were treated primarily only by marginal excision. Following re-excision two patients have remained disease-free until the present, the other four of these six patients had a second recurrence. Three of these four patients with a second relapse were successfully treated by excision. Although six patients out of 20 had 13 recurrences at the site of primary therapy, no patient developed lymph node or distant metastases. To date, none of the remaining 14 patients has had recurrence, all having been treated by excision of from 1 to 5 cm, the mean follow-up time was 8.75 years. Based on our data, the high recurrence rate after marginal surgical treatment implies an initial radical resection.     

Mohs micrographic surgery: established uses and emerging trends

Mohs micrographic surgery is a surgical technique that seeks to ensure the clearance of cutaneous tumors while maximizing normal tissue conservation. This is accomplished through the sequential removal of thin layers of tissue in which the entire peripheral and deep margins are examined for residual tumor. This approach appears to be superior to conventional surgical excision in the treatment of basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), the two most common cancers of the skin. Its efficacy in treating BCC and SCC has led clinicians to explore the role of Mohs micrographic surgery in the management of less common cutaneous neoplasms, such as melanoma, Merkel cell carcinoma, dermatofibrosarcoma protuberans, extramammary Paget's disease, and microcystic adnexal carcinoma.     

Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities.

AIMS: Dermatofibrosarcoma protuberans is a rare condition which is frequently misdiagnosed at presentation, resulting in a high incidence of local recurrence due to inadequate resection. The archives of the Department of Orthopaedics at the University of Florida were analysed to investigate the natural history and results of treatment for this tumour. METHODS: Between 1975 and 1996, 35 cases of DFSP were treated at the University of Florida. Of these, one was treated primarily, five were treated for local recurrence, 17 had tumour bed excisions following inadequate primary excisions elsewhere and 12 had tumour bed excisions following inadequate resection of local recurrences elsewhere. The data were analysed to assess the impact of age, gender, duration of symptoms, tumour site and size, surgical margin, number of operations and adjuvant treatments on survival and local recurrence outcomes. RESULTS: Complete follow-up was available for 34 patients. Mean follow-up was 58 months (range 12-144 months). Thirty-three patients remain alive and disease-free. One patient died of unrelated causes. The margins obtained were wide in 28 patients, marginal in six and intralesional in one. Of the seven patients with inadequate surgical margins, four received adjuvant radiation therapy and remain disease-free. No patient with an adequate margin developed a local recurrence, but there were three local recurrences in the patients with an inadequate margin who did not receive adjuvant radiation therapy (local recurrence rate: 8%). No patient developed lymphatic or distant metastasis. Local recurrences were more likely to be classified Stage IB (17/17) than primary tumours (1/18) (P<0.001). Local recurrence was more likely where the surgical margin was less than 2.5 cm from the lesion. CONCLUSIONS: Dermatofibrosarcoma protuberans is a low-grade tumour that has a high potential for local recurrence unless it can be completely excised. The overall rate of local recurrence in referred patients in this series was 20/35 cases (57%). All occurred after inadequate margins at previous surgery in other institutions. Revision surgery in these patients showed a local recurrence rate of 8%. To avoid extensive surgery for recurrences, initial treatment should be by wide excision incorporating the underlying deep fascia and a cuff of 2.5-3 cm of normal skin tissue. Radiation therapy provides a useful adjunct where adequate margins cannot be obtained.     

The Role of Radiation Therapy in the Management of Dermatofibrosarcoma Protuberans

Purpose: To evaluate the outcome for dermatofibrosarcoma protuberans treated with conservation surgery and radiation therapy.Methods and Materials: A retrospective review was performed of 19 consecutive patients with pathologically confirmed dermatofibrosarcoma protuberans who received radiation as an adjuvant to surgical resection.Results: The patients ages ranged from 19–76 years (median, 40 years); 12 were men. Lesions were located on the trunk in 8, in the head and neck area in 7, and in an extremity in 4. Tumor size ranged from 1.2 to 15 cm (median, 4 cm). Ten patients had at least 1 prior recurrence following earlier resection. Two patients received preoperative radiation to 50 Gy in 5 weeks. Sixteen patients underwent resection followed by radiation (6 of these had positive resection margins). In another patient, the tumor regrew rapidly after resection and definitive radiation was delivered for gross disease. The 6 patients with positive microscopic margins received a median dose of 60 Gy, as did the 10 with negative margins. The 1 patient with gross disease received 65 Gy. At a median follow-up of 6 years, the only patient to develop local recurrence was treated with definitive radiotherapy for gross disease. Actuarial local control was 95% at 10 years.Conclusion: Dermatofibrosarcoma protuberans is a radioresponsive tumor and radiation to doses of 50–60 Gy should be considered as an adjuvant to resection if margins are positive. Combined conservation resection and postoperative radiation should also be considered for situations where adequate wide excision alone would result in major cosmetic or functional deficits.     

Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years. METHODS: The records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution. RESULTS: Sixty-three DFSP lesions were removed from 62 patients. At a median follow-up of 4.4 years, no local or distant recurrences were detected in any patient. Forty-three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety-five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty-five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%). CONCLUSIONS: Wide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution.     

隆突性皮肤纤维肉瘤18例临床分析

目的　探讨隆突性皮肤纤维肉瘤 (DFSP)术后复发的因素。方法　回顾性分析 18例隆突性皮肤纤维肉瘤诊治过程及复发情况。结果　 7例隆突性皮肤纤维肉瘤行扩大根治切除术 ,复发率为 43 % ;11例行扩大切除加术后局部放疗 ,复发率为 18%。同时DFSP复发与组织学分级显著相关。结论　对隆突性皮肤纤维肉瘤认识不足 ,首次手术切除不彻底 ,扩大根治不规范 ,术后未行放疗及瘤细胞分化程度低是其复发的主要因素。     

74例隆突型皮肤纤维肉瘤的治疗与预后分析

Objective： To study treatment and prognostic factor in 74 patients with dermatofibrosarcoma protuberans. Methods： From August 1990 to November 1999, 74 patients with dermatofibrosarcoma protuberans （DFSP） confirmed by pathology were treated in Cancer Hospital of Sun Yat-sen University. 72 cases were given wide excision and 2 cases were given local excision. All of 74 cases, 52 cases had surgical resection alone, and 22 cases had surgical resection combined with radiotherapy. Total dose of radiotherapy was 50-70 Gy. Results： The rate of recurrence was 36.1% for all patients. The 5-year recurrence-free survival （RFS） rate was 66%. The 5-year recurrence-free survival rates for resection alone and combined with radiotherapy were 58% and 90%, respectively （P=-0.0187）. The 5-year recurrence-free survival rates for positive microscopic margins and negative microscopic margins were 57% and 75%, respectively （P=0.0468）. Conclusion： Post-operation radiotherapy is an effective treatment to decrease the recurrence rate for the patients with positive microscope, or the patients without suitable surgical treatment.     

Desmoid tumour: a pleomorphic lesion.

BACKGROUND: Desmoid tumour (DT) is an uncommon locally invasive non-metastasizing neoplastic lesion. The aetiology of this tumour is unknown and its treatment is controversial. Twelve cases of DT are presented and the literature is reviewed. METHODS: Twelve cases of DT treated at our institution during a 3.5-year period are analysed and the literature reviewed. Ten patients were referred with a primary tumour, one with local recurrence and one patient with a second primary desmoid tumour. One patient had multiple mesenteric DT (familial adenomatous polyposis coli-FAP), and in the remaining 11 patients the tumour was located in the abdominal wall in four, at an extremity in three, in the upper back in two patients, in the pelvis in one and retroperitoneally in one. RESULTS: The largest mesenteric DT was marginally excised en bloc with total jejunectomy. In the remaining 11 DT, complete excision to microscopically tumour-free margins was possible in nine cases and to microscopically involved margins in two cases. At a mean follow-up of 22 months (range 7-38 months), one patient was alive with stable disease (Gardner's syndrome), 10 patients were alive and free of recurrence and one patient (9%) developed local recurrence which was re-excised-she is disease-free 10 months later. CONCLUSIONS: Complete excision is the main modality of treatment for primary and recurrent DT. This is feasible in most cases except for tumours involving the base of the bowel mesentery. Surgical resection alone achieved local control of the tumour in most of the patients in this series (92%).     

Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans.

PURPOSE: Dermatofibrosarcoma protuberans is caused by activation of the platelet-derived growth factor B (PDGFB) receptor, a transmembrane tyrosine kinase. We investigated the response of dermatofibrosarcoma protuberans to the tyrosine kinase inhibitor imatinib mesylate. PATIENTS AND METHODS: A patient with unresectable, metastatic dermatofibrosarcoma protuberans received imatinib mesylate (400 mg bid). Response to therapy was assessed by [18F]fluorodeoxyglucose (FDG) positron emission tomography, magnetic resonance imaging, and histopathologic and immunohistochemical evaluation. RESULTS: The patient was treated for 4 months with imatinib mesylate. The hypermetabolic uptake of FDG fell to background levels within 2 weeks of treatment, and the tumor volume shrank by over 75% during the 4 months of therapy, allowing for resection of the mass. There was no residual viable tumor in the resected specimen, indicating a complete histologic response to treatment with imatinib mesylate. CONCLUSION: Imatinib mesylate is highly active in dermatofibrosarcoma protuberans. The dramatic response seen in this patient demonstrates that inhibition of PDGFB receptor tyrosine kinase activity can significantly impact viability of at least one type of solid tumor.     

Dermatofibrosarcome protuberans récidivant du sein: à propos d’un cas

The dermatofibrosarcoma protuberans (DFSP) is a sarcoma of low rank developing slowly from the fibroblasts of the dermis. The main characteristic of this tumour is its big potential for local recurrence. We report the case of a patient aged 44 presenting a DFSP of the breast that relapsed on two occasions. The remaining extension was negative. She benefited from a large timorous excision. The mammary localization of a DFSP is rare. The diagnosis is difficult. The therapeutic objective of DFSP is to reach a possible most complete surgical resection; the conventional surgical excision must let healthy margins (of 3 to 5 cm) with the inconvenience of the substance loss. The technique of Moh, verifying the limits of excision in preoperative, can palliate this inconvenience. The risk of recurrence is low when the resection margins are healthy. Radiotherapy as adjuvant treatment is very useful when the margins are not sufficient. The surveillance must be prolonged.     

Penile tumors: their management by Mohs micrographic surgery

Penile tumors represent a difficult group of neoplasms requiring effective and curative treatment while minimizing tissue loss to prevent cosmetic and functional deformity. Over the past 6 years, we have treated 20 patients with penile cancer utilizing the fresh tissue technique of Mohs micrographic surgery. Tumors were excised with an average of 2.25 stages. Most defects (80%) were allowed to heal by second intention. Since surgery, four patients have developed metastatic disease in their regional lymphatic system, and one patient has died from metastatic spread. One patient has developed local recurrence. Micrographic surgery is a very useful treatment modality for patients with penile tumors. Patients with SCC of the penis should be considered for elective regional lymph node biopsy and/or dissection in conjunction with micrographically controlled excision of the primary tumor.     

3D-Histological evaluation of surgery in dermatofibrosarcoma protuberans and malignant fibrous histiocytoma: Differences in growth patterns and outcome

Aims

To evaluate the microscopic growth pattern of dermatofibrosarcoma protuberans (DFSP) and malignant fibrous histiocytoma (MFH) and the long-term outcome using 3D-histologic surgery with paraffin sections to cover complete margins and to detect subclinical spreads very sensitively.

Methods

One hundred and one patients have been included comprising 70 DFSP, 31 MFH. Data from 87 patients treated since 1992 were collected prospectively.

Results

Mean clinical tumor-size was 45 mm, mean histological tumor size 65 mm. A mean excision margin of 19 mm achieved negative margins. The histological infiltration shows an asymmetrical pattern with horizontal or vertical extension either cord-, sector- or multiple-like up to 70 mm in length, detectable by 3D-histology. Age and localization differed significantly between DFSP and MFH lesions. MFH tumors had a significantly deeper infiltration than DFSP. The mean follow up was 60 months. In 70 patients with DFSP one local recurrence after 62 months occurred, but no metastasis. 31 patients with MFH developed 8 local recurrences, and 4 metastases (lymph nodes and/ or lungs); 3 of them died of the disease, all 3 had a postoperative status of R1 (p = 0.001).

Conclusions

There are significant differences in growth pattern and clinical outcome between DFSP and MFH. DFSP can be cured by surgery following 3D-histology with paraffin sections. MFH is significantly more malignant. After local R0-resection proofed by 3D-histology higher cure rates can be achieved.     

Intracranial recurrence and distant metastasis of scalp dermatofibrosarcoma protuberans

Only few cases of scalp dermatofibrosarcoma protuberans with intracranial and distant metastasis have been reported. Here we report a case of scalp dermatofibrosarcoma protuberans with frequent local recurrence, intracranial invasion and with distant lung metastasis during 6 years of treatment. We would like to emphasize difficulties in surgical treatment of such invasive and locally recurrent tumors of scalp, and necessity to understand new molecular pathogenesis of dermatofibrosarcoma protuberans and potential treatment strategy with imatinib for patients with surgically untreatable disease. Close surveillance of patients with scalp dermatofibrosarcoma is necessary due recurrence nature of tumor.     

Mohs surgery is the treatment of choice for recurrent (previously treated) basal cell carcinoma

We reviewed all studies (since 1945) reporting recurrence rates for treatment of recurrent (previously treated) basal cell carcinomas (BCC) using surgical excision, radiotherapy, cryotherapy, curettage and electrodesiccation, and Mohs micrographic surgery. The 5-year recurrence rate for Mohs micrographic surgery is 5.6%. The recurrence rate for non-Mohs modalities of 19.9% is nearly four times higher. Individual recurrence rates for the non-Mohs modalities are 17.4% for surgical excision, 40.0% for curettage and electrodesiccation, and 9.8% for radiation therapy. There are no studies reporting 5-year data for cryotherapy. However, the recurrence rate is 13.0% for cryotherapy when the follow-up period is less than five years. The data support the following conclusions: (1) Mohs surgery is the treatment of choice for recurrent BCC; (2) if the patient is not a surgical candidate and the lesion is small, radiation therapy is an alternative that offers a better chance for cure than the other non-Mohs modalities; and (3) curettage and electrodesiccation should not be used to treat recurrent basal cell carcinoma.     

Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior

PURPOSE OF REVIEW: Dermatofibrosarcoma protuberans is a low-grade malignancy of the skin and subcutaneous tissues with low potential for the development of distant metastases. This tumor is characterized by infiltrative growth, and a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the gene platelet-derived growth factor B gene on chromosome 22. This review will examine recent data confirming the central role of surgery in managing this disease and new findings for the application of molecularly targeted therapy in patients with unresectable disease. RECENT FINDINGS: The largest published series of dermatofibrosarcoma protuberans cases has reported that surgery can lead to excellent local control in dermatofibrosarcoma protuberans in over 95% of cases. In patients with metastatic disease, or with locally advanced disease not suitable for surgical excision, inhibition of platelet-derived growth factor receptors with imatinib (Gleevec) can lead to striking clinical results, including complete responses in some patients. SUMMARY: The identification of platelet-derived growth factor B as a near universal translocation partner in chromosomal rearrangements in dermatofibrosarcoma protuberans has led to the successful application of therapy targeted at platelet-derived growth factor receptors. Although wide surgical excision remains the standard of care, patients with locally advanced disease not suitable for wide surgical excision or with metastatic disease can be managed with the platelet-derived growth factor receptors inhibitor imatinib with high probability of response.     

Primary squamous cell carcinoma of the thyroid.

AIMS: To investigate the clinical features and treatment protocol of squamous cell carcinoma of the thyroid (SCCT). METHOD: Clinical records of four SCCT patients treated during 1985--99 were retrospectively reviewed and evaluated. RESULTS: Two patients who underwent surgical excision plus radiotherapy died of local tumour recurrence, 6 and 13 months, respectively, post-operatively. One who accepted surgery only died 4 months later of respiratory distress. The fourth patient who had radical surgery coupled with radiotherapy and chemotherapy was disease-free at 26-month follow-up. CONCLUSION: SCCT is a very rare but highly malignant carcinoma. Early diagnosis and an aggressive radical surgery portend a better prognosis. Radiotherapy and chemotherapy are secondary applicable methods for the patients with certain condition.     

Dermatofibrosarcome de Darier-Ferrand: deux cas à localisation cervicofaciale

The authors illustrate the diagnostic and therapeutic difficulties encountered with Darier and Ferrand dermatofibrosarcoma from 2 cases report. Patients were 40 and 31 years old. In the first case, the tumour had been evolving for 4 years and occupied the submandibular region. In the second case, it had been evolving for 14 years and was localized in the parotido-submandibular region. The two patients benefitted from at least a biopsy or a partial surgical gesture before admission in our service. Surgical excision was wide. Both patients are alive after 4 and 2 years without recurrence and metastasis. Darier and Ferrand dermatofibrosarcoma is a cutaneous tumour of local malignancy, often diagnosed after several recurrences. The best treatment is surgery and consists in a wide and deep excision sacrificing healthy tissue.     

Clinical presentation and long-term outcome of pure myoepithelial carcinoma of the breast.

INTRODUCTION: Pure myoepithelial carcinoma of the breast is a rare tumour of controversial histogenesis. Little is known about its natural history and long-term outcome following treatment. METHODS: All patients with pure myoepithelial carcinoma treated at our institution between 1970 and 2001 were studied with respect to pathological features, outcome and prognosis. RESULTS: Six patients were identified. The median age was 60 (40-66) years and median follow-up was 34.5 months (range 14-76) months. Four tumours were T1 and one was T2 (one tumour size unknown). There were two moderately differentiated and three well-differentiated tumours (grade could not be assessed in one patient). Oestrogen and progesterone receptor could be assessed in five patients and all were negative. Primary treatment was wide local excision with clear radial margins. Lymph node assessment was negative in all patients. One patient received adjuvant radiotherapy. Three patients developed local recurrence at 15, 38 and 50 months and two patients developed distant metastasis at 30 and 79 months. The local recurrences were treated by further excision but two patients developed distant metastasis at intervals of 15 and 26 months, respectively. Two patients have died of the disease and four remain well. The 2-year and 5-year survival was 88% (SE, 6) and 55% (SE, 16), respectively. Large tumour size is a prognostic indicator of poor outcome. CONCLUSION: Pure myoepithelial carcinoma of the breast adopts an aggressive clinical course with an outcome comparable to poorly differentiated adenocarcinoma of the breast.