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Objectives
There is limited literature on the optimal treatment of sarcoma arising in the scalp. This study evaluates local relapse (LR) and survival outcomes of patients with scalp sarcoma treated at a provincial cancer care institution.
Methods
A retrospective review of 95 patients with a primary diagnosis of scalp sarcoma referred from 1990-2015...
Context in source publication
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Citations
... Several studies have examined the risk benefit ratio associated with preoperative radiation of angiosarcoma of the scalp and its impact on survival [13][14][15]. In a meta-analysis by Shin et al. [16], it was found that radiation therapy did not have a significant effect on survival in head and neck angiosarcoma. ...
Background: Oncologic resection of the scalp confers several obstacles to the reconstructive
surgeon dependent upon patient-specific and wound-specific factors. We aim to describe our ex-
periences with various reconstructive methods, and delineate risk factors for coverage failure and
complications in the setting of scalp reconstruction.
Methods: A retrospective chart review was conducted, examining patients who underwent re-
section of fungating scalp tumors with subsequent soft-tissue reconstruction from 2003 to 2019.
Patient demographics, wound and oncologic characteristics, treatment modalities, and outcomes
were recorded and analyzed.
Results: A total of 189 patients were appropriate for inclusion, undergoing a range of reconstruc-
tive methods from skin grafting to free flaps. Thirty-three patients (17.5%) underwent preopera-
tive radiation. In all, 48 patients (25.4%) suffered wound site complications, 25 (13.2%) under-
went reoperation, and 47 (24.9%) suffered from mortality. Preoperative radiation therapy was an
independent risk factor for wound complications (odds ratio [OR], 2.85; 95% confidence interval
[CI], 1.1–7.3; p = 0.028) and reoperations (OR, 4.45; 95% CI, 1.5–13.2; p = 0.007). Similarly, the
presence of an underlying titanium mesh was an independent predictor of wound complications
(OR, 2.49; 95% CI, 1.1–5.6; p= 0.029) and reoperations (OR, 3.40; 95% CI, 1.2–9.7; p= 0.020). Both
immunosuppressed status (OR, 2.88; 95% CI, 1.2–7.1; p= 0.021) and preoperative radiation thera-
py (OR, 3.34; 95% CI, 1.2–9.7; p= 0.022) were risk factors for mortality.
Conclusion: Both preoperative radiation and the presence of underlying titanium mesh are inde-
pendent risk factors for wound site complications and increased reoperation rates following onco-
logic resection and reconstruction of the scalp. Additionally, preoperative radiation, along with an
immunosuppressed state, may predict patient mortality following scalp resection and reconstruction.
... Pleomorphic dermal sarcoma (PDS) and atypical fibroxanthoma (AFX) are uncommon mesenchymal neoplasms that mainly present in sun-damaged regions of elderly patients and share histopathologic and immunohistochemical (IHC) features [1,2]. PDS displays more aggressive clinical behavior than AFX and has a higher rate of recurrences, with some studies reporting an average of 20-30% rates of local recurrence and 10-20% rates of distant recurrences [2][3][4]. PDS is most often located in the head and neck region and has a predilection for men, with one study reporting a 7:1 male to female ratio and another study reporting a 3.7:1 ratio [2,5,6]. Epidermal ulceration and bleeding are common along with periods of rapid growth. ...
... PDS is a more malignant cancer than AFX, with higher rates of local recurrence and distant metastases [2,39]. Local recurrences are observed in 20-30% of patients and 10-20% of patients develop metastatic disease [2][3][4]. One major risk of recurrence is incomplete resection of tumors during primary surgery. ...
Pleomorphic dermal sarcoma is an uncommon mesenchymal neoplasm that shares clinical, immunohistochemical, and histopathologic features with atypical fibroxanthoma but with additional aggressive histopathologic features. This tumor is not well documented in the literature, its clinical features are poorly characterized, and diagnosis is still by exclusion. To better understand this disease, we performed a comprehensive systematic review and included clinical, immunohistochemical, and histopathologic data of five patients (median age: 81) from our own institution. Elderly men are typically affected more than women and the tumor presents as an exophytic growth on sun-damaged regions of the head and scalp, although our cohort had three men and two women. Histologically, the tumors display brisk mitotic activity with asymmetrical lesions; pleomorphic spindle cells; and large, multinucleated giant cells with vacuolated cytoplasm arranged in fascicular patterns. Eighty percent of patients displayed deep tissue invasion and 40% displayed perineural invasion. Immunohistochemical stains for CD10 and CD68 were positive in 60%, and all patients tested were negative for S-100, PanCK, desmin, MART-1, SOX-10 CD-34, P40, SMA, and CK-5/6. Recurrence and further clinical data were not collected due to loss of follow-up. Although there are no standardized treatment guidelines, wide local excision is recommended. The consensus is that pleomorphic dermal sarcoma has a more aggressive disease course than atypical fibroxanthoma with poorer outcomes. Our study creates the first comprehensive treatment recommendations in the literature for better risk stratification, prognoses, and outcomes.
... There is only limited evidence that neoadjuvant radiotherapy for the treatment of PDS on the scalp is effective [18,27,28]. Within the reported cases, we found radiotherapy primarily as an adjunctive treatment. ...
Simple Summary
Atypical fibroxanthoma and pleomorphic dermal sarcoma represent two tumors on the spectrum of a rare dermal sarcoma entity. Close clinical presentation and nearly identical histologic features but distinct prognoses make proper treatment strategies challenging. We performed a retrospective analysis of 32 patients with AFX or PDS in the scalp to provide guidance regarding the extent of radical excision to achieve stable oncological outcomes and whether radical tumor resection on the scalps required complex soft-tissue reconstruction. Compared to AFX, PDS shows a more aggressive growth pattern with frequent satellite metastases and distant metastases. These require extensive resections for local control to achieve long-term remission in most PDS patients. Despite the limited elasticity of the scalp, plastic reconstructive procedures can obtain reliable soft tissue reconstruction, even for complete scalp defects. Due to their rarity, managing these tumors requires an interdisciplinary setting in a specialized sarcoma center.
Abstract
Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are two distinct designations for a rare dermal sarcoma entity. These tumors arise predominantly in the sun-damaged skin of elderly patients. Although both AFX and PDS have a similar clinical presentation and nearly identical genetic features, they significantly differ in prognosis. Here we present a retrospective single-center chart review analyzing the outcomes of patients treated for dermal sarcoma. The radicality of the tumor-resection extent and soft-tissue reconstructive options were assessed. Patients between January 2010 and August 2021 were included. We recorded resection margins, tumor recurrence, overall survival, number of operations until complete tumor resection, and reconstructive procedures; any complications were recorded. Furthermore, we analyzed a subgroup of patients with satellite metastases. A total of 32 patients met the inclusion criteria (30 male, 2 female, median age of 77.5 years (interquartile range (IQR) 74–81)). Histopathology revealed AFX in 14 patients and PDS in 18 patients. Margin-free resection was achieved in 31 cases, and 27 patients were remission free over the reported period. The local recurrence rate was 5, and distant metastasis was detected in four cases. Of all the PDS cases, nine presented with satellite metastasis. No AFX had satellite metastases. Due to their rarity, managing these tumors requires an interdisciplinary setting in a specialized sarcoma center.
... Limited invasion of the brain parenchyma can be treated with excision but large lesions are often unresectable and are associated with morbidity. Even if they are resected with negative margins they are associated with a high recurrence rate of around 20%. 8 There are no specific treatment guidelines for STS of scalp. Adjuvant radiation has not shown to reduce the rate of recurrence. ...
Scalp soft tissue sarcomas (STS) are very rare accounting for less than 0.1% of all malignancies. We report a rare clinical image of advanced stage soft tissue sarcoma of the scalp. A 65 year woman had presented to the surgical department with complaints of a rapidly growing swelling over the scalp for three months. On examination there was huge 20 x 20 cm swelling over the scalp in the left temporoparietal region with variegated consistency. Computed tomography of head revealed a large soft tissue mass with necrosis invading the bone and underlying brain parenchyma. Histopathological finding from core needle biopsy revealed pleomorphic sarcoma. STS are highly malignant tumors which should be diagnosed and treated using multimodality approach. Recurrences are common even after complete resection and prognosis is poor.
... Hence, a systematic search in PubMed of 'pleomorphic dermal sarcoma'" was conducted in April 2021, netting 134 articles, from which 15 were longitudinal studies that provided outcomes specific to PDS (table 1). [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Reported characteristics and outcomes (besides age) were pooled via randomeffects meta-analysis using the generalised linear mixed model with logit transformation, along with Clopper-Pearson CIs for individual studies. 17 Heterogeneity was assessed via the I 2 statistic and the likelihood-ratio test. ...
... The optimal local treatment for this disease has not been determined and could include surgery, radiation therapy, or both modalities. Given the rarity of scalp and face angiosarcoma, prospective studies showing the benefit of radiation therapy are lacking, but several retrospective studies have demonstrated an improved local recurrence and survival with radiation treatment [6,[8][9][10]. ...
... Specifically, the 3-year local control rate with surgery alone or radiation alone was 30%, while the 3-year local control rate with surgery and radiation was 95% [6]. The British Columbia experience showed a trend to improved local relapse-free survival with surgery, followed by radiation vs. surgery alone (70% vs. 40%) [10]. ...
Purpose:
Angiosarcoma is a sub-type of soft tissue sarcoma, often presenting as a multifocal or diffuse disease process with poor prognosis. This study presents outcomes of a single institution cohort of patients with angiosarcoma of the scalp and face following treatment with multimodality therapy, including high-dose-rate surface applicator (HDR-SA) brachytherapy, and represents the largest cohort utilizing this therapeutic approach.
Material and methods:
Twenty patients with primary or recurrent angiosarcoma of the face or scalp were treated with HDR-SA brachytherapy between 2003-2018, with clinical characteristics and outcomes collected from medical records and used to identify prognostic features.
Results:
Median follow-up was 45 months. Patients treated with HDR-SA brachytherapy had a 4-year local control rate of 63%, a 4-year progression-free survival (PFS) rate of 20%, and a 4-year overall survival rate of 54%. Disease features associated with worse loco-regional control (LRC) included location on the scalp (vs. face, p = 0.04) and tumor size ≥ 5 cm (p = 0.0099). Outcomes after HDR-SA brachytherapy for salvage therapy vs. HDR-SA brachytherapy as a component of an initial treatment approach were also significantly different, with worse LRC (p = 0.0084) and worse overall survival (OS) (p = 0.0019) in a setting of salvage therapy.
Conclusions:
Local control rates following HDR-SA brachytherapy for scalp or face angiosarcoma are moderate and similar to what is described in the literature using a variety of local control treatment modalities. Smaller tumors and those involving the face rather than scalp had better outcomes. PFS rates were poor and there is a pressing need for treatment intensification and novel therapeutic options.
... Several studies have examined the risk benefit ratio associated with preoperative radiation of angiosarcoma of the scalp and its impact on survival [13][14][15]. In a meta-analysis by Shin et al. [16], it was found that radiation therapy did not have a significant effect on survival in head and neck angiosarcoma. ...
Background:
Oncologic resection of the scalp confers several obstacles to the reconstructive surgeon dependent upon patient-specific and wound-specific factors. We aim to describe our experiences with various reconstructive methods, and delineate risk factors for coverage failure and complications in the setting of scalp reconstruction.
Methods:
A retrospective chart review was conducted, examining patients who underwent resection of fungating scalp tumors with subsequent soft-tissue reconstruction from 2003 to 2019. Patient demographics, wound and oncologic characteristics, treatment modalities, and outcomes were recorded and analyzed.
Results:
A total of 189 patients were appropriate for inclusion, undergoing a range of reconstructive methods from skin grafting to free flaps. Thirty-three patients (17.5%) underwent preoperative radiation. In all, 48 patients (25.4%) suffered wound site complications, 25 (13.2%) underwent reoperation, and 47 (24.9%) suffered from mortality. Preoperative radiation therapy was an independent risk factor for wound complications (odds ratio [OR], 2.85; 95% confidence interval [CI], 1.1-7.3; p = 0.028) and reoperations (OR, 4.45; 95% CI, 1.5-13.2; p = 0.007). Similarly, the presence of an underlying titanium mesh was an independent predictor of wound complications (OR, 2.49; 95% CI, 1.1-5.6; p= 0.029) and reoperations (OR, 3.40; 95% CI, 1.2-9.7; p= 0.020). Both immunosuppressed status (OR, 2.88; 95% CI, 1.2-7.1; p= 0.021) and preoperative radiation therapy (OR, 3.34; 95% CI, 1.2-9.7; p= 0.022) were risk factors for mortality.
Conclusion:
Both preoperative radiation and the presence of underlying titanium mesh are independent risk factors for wound site complications and increased reoperation rates following oncologic resection and reconstruction of the scalp. Additionally, preoperative radiation, along with an immunosuppressed state, may predict patient mortality following scalp resection and reconstruction.
... Given the rarity of scalp angiosarcoma, prospective studies are lacking, but several retrospective studies indicate that patients who receive multimodality care appear to have lower local recurrence and improved survival. [1][2][3][4][5] The study by Kang et al. retrospectively analyzed 40 consecutive patients with scalp angiosarcoma at their institution. 6 Interestingly, they found that regional lymph node (LN) metastases were seen in over 50% of patients with scalp angiosarcomas during their disease course, which is higher than previously reported. ...
... Several studies have examined the risk benefit ratio associated with preoperative radiation of angiosarcoma of the scalp and its impact on survival [13][14][15]. In a meta-analysis by Shin et al. [16], it was found that radiation therapy did not have a significant effect on survival in head and neck angiosarcoma. ...
Introduction:
Soft-tissue reconstruction of the scalp has traditionally been challenging in oncologic patients. Invasive tumors can compromise the calvarium, necessitating alloplastic cranioplasty. Titanium mesh is the most common alloplastic material, but concerns of compromise of soft-tissue coverage have introduced hesitancy in utilization. The authors aim to identify prognostic factors associated with free-flap failure in the context of underlying titanium mesh in scalp oncology patients.
Methods:
A retrospective review (2010-2018) was conducted at a single center examining all patients following oncologic scalp resection who underwent titanium mesh cranioplasty with free-flap reconstruction following surgical excision. Patient demographics, comorbidities, ancillary oncological treatment information were collected. Operative data including flap type, post-operative complications including partial and complete flap failure were collected.
Results:
A total of 16 patients with 18 concomitant mesh cranioplasty and free-flap reconstructions were identified. The majority of patients were male (68.8%), with an average age of 70.5 years. Free-flap reconstruction included 15 ALT flaps (83.3%), 2 latissimus flaps (11.1%), and one radial forearm flap (5.5%). There were three total flap losses in two patients. Patient demographics and comorbidities were not significant prognostic factors. Additionally, post-operative radiation therapy, ancillary chemotherapy, oncological histology, tumor recurrence, and flap type were not found to be significant. Pre-operative radiotherapy was significantly associated with flap failure (P < 0.05).
Conclusion:
Pre-operative radiotherapy may pose a significant risk for free-flap failure in oncologic patients undergoing scalp reconstruction following mesh cranioplasty. Awareness of associated risk factors ensures better pre-operative counseling and success of these reconstructive modalities and timing of pre-adjuvant treatment.
