Wide Surgical Resection Research Articles

Evaluation of the efficacy and safety of toceranib phosphate in cats with macroscopic mammary adenocarcinoma.

Mammary tumours in cats are biologically aggressive. The standard of care relies upon wide surgical resection. Chemotherapy has been described in the macroscopic disease setting; however, limited efficacy has been shown. The aim of this study was to assess the efficacy of toceranib phosphate in macroscopic feline mammary tumours (FMTs). A total of 17 cats with cytologically or histopathologically confirmed mammary adenocarcinoma (gross disease) were prospectively enrolled. Toceranib phosphate was administered at a median dose of 2.77 mg/kg (range 2.3-3.2) PO q48 h. No corticosteroids or non-steroidal anti-inflammatory drugs (NSAIDs) were administered. Toxicity was graded according to Veterinary Cooperative Oncology Group-Common Terminology Criteria for Adverse Events (VCOG-CTCAE) v1.1 criteria. The response was assessed after 1 month, following Response Evaluation Criteria In Solid Tumours (RECIST) criteria. Toxicity was observed in eight cats, with most instances being grade 1 or 2, which were managed with supportive care. Only one cat experienced grade 3 toxicity (anorexia), which resolved after a dose reduction. Clinical benefit was seen in 12 (64.7%) cats and an objective response was seen in six (35.2%) cats. One cat experienced complete response, five had partial response, six had stable disease and five had progressive disease. One cat showed distant progression (malignant pleural effusion) despite continued partial remission of the primary tumour. The median progression-free survival and median overall survival time were 91 days (range 30-158) and 145 days (range 31-234), respectively. Toceranib phosphate showed clinical benefit and a good safety profile in advanced or recurrent FMTs, offering a new alternative in the treatment of this disease; however, further prospective and randomised studies are required to further assess its efficacy. Interestingly, one cat developed distant metastases while the primary tumour showed partial response, suggesting that primary tumour and metastatic disease may not sustain the same sensitivity to toceranib.

Imaging-Based Deep Learning for Predicting Desmoid Tumor Progression.

Desmoid tumors (DTs) are non-metastasizing and locally aggressive soft-tissue mesenchymal neoplasms. Those that become enlarged often become locally invasive and cause significant morbidity. DTs have a varied pattern of clinical presentation, with up to 50-60% not growing after diagnosis and 20-30% shrinking or even disappearing after initial progression. Enlarging tumors are considered unstable and progressive. The management of symptomatic and enlarging DTs is challenging, and primarily consists of chemotherapy. Despite wide surgical resection, DTs carry a rate of local recurrence as high as 50%. There is a consensus that contrast-enhanced magnetic resonance imaging (MRI) or, alternatively, computerized tomography (CT) is the preferred modality for monitoring DTs. Each uses Response Evaluation Criteria in Solid Tumors version 1.1 (RECIST 1.1), which measures the largest diameter on axial, sagittal, or coronal series. This approach, however, reportedly lacks accuracy in detecting response to therapy and fails to detect tumor progression, thus calling for more sophisticated methods. The objective of this study was to detect unique features identified by deep learning that correlate with the future clinical course of the disease. Between 2006 and 2019, 51 patients (mean age 41.22 ± 15.5 years) who had a tissue diagnosis of DT were included in this retrospective single-center study. Each had undergone at least three MRI examinations (including a pretreatment baseline study), and each was followed by orthopedic oncology specialists for a median of 38.83 months (IQR 44.38). Tumor segmentations were performed on a T2 fat-suppressed treatment-naive MRI sequence, after which the segmented lesion was extracted to a three-dimensional file together with its DICOM file and run through deep learning software. The results of the algorithm were then compared to clinical data collected from the patients' medical files. There were 28 males (13 stable) and 23 females (15 stable) whose ages ranged from 19.07 to 83.33 years. The model was able to independently predict clinical progression as measured from the baseline MRI with an overall accuracy of 93% (93 ± 0.04) and ROC of 0.89 ± 0.08. Artificial intelligence may contribute to risk stratification and clinical decision-making in patients with DT by predicting which patients are likely to progress.

Photoimmunotheranostics of epithelioid sarcoma by targeting CD44 or EGFR

Epithelioid sarcoma (ES) is a rare soft tissue neoplasm with high recurrence rates. Wide surgical resection remains the only potential curative treatment. ES presents most commonly on the fingers, hands and forearm, making light-based cancer cell-targeted therapies such as near-infrared photoimmunotherapy (NIR-PIT) that is target-specific, but with limited penetration depth, suitable for ES treatment. We established that CD44 and EGFR were overexpressed in ES patient samples and in the VA-ES-BJ human ES cell line. NIR-PIT of VA-ES-BJ cells using antibody photosensitizer conjugates, prepared by conjugating a CD44 or EGFR monoclonal antibody to the photosensitizer IR700, confirmed that NIR-PIT with both conjugates resulted in cell death. Neither treatment with NIR light alone nor treatment with the conjugates but without NIR light were effective. CD44-IR700-PIT resulted in greater cell death than EGFR-IR700-PIT, consistent with the increased expression of CD44 by VA-ES-BJ cells. In tumors, EGFR-IR700 exhibited a higher tumor-to-normal ratio, as determined by in vivo fluorescence imaging, and a higher anti-tumor growth effect, compared to CD44-IR700. No antitumor effect of the EGFR antibody or the photosensitizer conjugate alone was observed in vivo. Our data support evaluating the use of EGFR-IR700-PIT in the management of ES for detecting and eliminating ES cells in surgical margins, and in the treatment of superficial recurrent tumors.

POSTOPERATIVE RADIOTHERAPY IN SALIVARY DUCTCARCINOMA: A CASE REPORT AND REVIEW OF THE LITERATURE

Salivary duct carcinoma(SDC) is a rare and aggressive tumor of the salivary glands that often occurs in men between the fifth and sixth decades of life. standard treatment is based on wide surgical resection of the primary tumor with lymph node dissection, followed by postoperative radiotherapy. The place of chemotherapy in the treatment of SDC remains unknown. In this publication, authors report a case of SDC of the left parotid gland. A 28-year-old male suffered from a painful neck mass associated with hearing loss that had been evolving for 6 months. The patient initially had a cervical CT scan and a parotid MRI, which showed a tumor of the left parotid gland. He initially had a total parotidectomy preserving the facial nerve with an ipsilateral neck dissection, as the intraoperative histological examination was in favor of malignancy. The final analysis of the surgical specimen concluded to a ductal carcinoma of the parotid with lymph node metastasis. Adjuvant radiation therapy has been indicated. The evolution was unfavorable due to the appearance of multisite metastases after one year.

The Effect of chemo- and radiotherapy on tumor necrosis in soft tissue sarcoma– does it influence prognosis?

BackgroundSoft tissue sarcomas (STSs) are a heterogeneous group of tumors. Wide surgical resection is standard, often combined with neoadjuvant chemotherapy, radiotherapy, or both. Studies have shown the predictive value of tumor necrosis in bone sarcoma (BS); however, the role of necrosis in STS after neoadjuvant therapies is still unclear. This study aimed to investigate the role of chemo- and radiotherapy in the formation of tumor necrosis and to evaluate the influence of tumor necrosis on overall survival and local recurrence-free survival. Data from BS patients and patients who did not receive neoadjuvant therapy were compared.MethodsA total of 779 patients with STS or BS were treated surgically. In all patients, tumor-specific factors such as type, size, or grading and the type of adjuvant therapy were documented. Local recurrence (LR), the diagnosis of metastatic disease, and survival during follow-up were evaluated.ResultsA total of 565 patients with STS and 214 with BS were investigated. In STS, 24.1% G1 lesions, 34.1% G2 lesions, and 41.8% G3 lesions were observed. Two hundred twenty-four of the patients with STS and neoadjuvant therapy had either radiotherapy (RTx) (n = 80), chemotherapy (CTx) (n = 93), or both (n = 51). Three hundred forty-one had no neoadjuvant therapy at all. In STS, tumor necrosis after neoadjuvant treatment was significantly higher (53.5%) than in patients without neoadjuvant therapy (15.7%) (p < 0.001). Patients with combined neoadjuvant chemo-/radiotherapy had substantially higher tumor necrosis than those with radiotherapy alone (p = 0.032). There was no difference in tumor necrosis in patients with combined chemo-/radiotherapy and chemotherapy alone (p = 0.4). The mean overall survival for patients with STS was 34.7 months. Tumor necrosis did not influence survival in a subgroup of G2/3 patients. In STS with no neoadjuvant therapy and grading of G2/3, the correlation between necrosis and overall survival was significant (p = 0.0248). There was no significant correlation between local recurrence (LR) and necrosis.ConclusionSTS shows a broad spectrum of necrosis even without neoadjuvant chemo- or radiotherapy. After CTx or/and RTx necrosis is enhanced and is significantly pronounced with a combination of both. There is a trend toward higher necrosis with CTx than with RTx. Grading substantially influences the necrosis rate, but necrosis in soft-tissue sarcoma following neoadjuvant therapy does not correlate with better survival or a lower local recurrence rate, as in bone sarcomas.

Genetically engineered mouse model of pleomorphic liposarcoma: Immunophenotyping and histologic characterization

IntroductionPleomorphic liposarcoma is a rare and aggressive subset of soft-tissue sarcomas with a high mortality burden. Local treatment largely consists of radiation therapy and wide surgical resection, but options for systemic therapy in the setting of metastatic disease are limited and largely ineffective, prompting exploration of novel therapeutic strategies and experimental models. As with other cancers, sarcoma cell lines and patient-derived xenograft models have been developed and used to characterize these tumors and identify therapeutic targets, but these models have inherent limitations. The establishment of genetically engineered mouse models represents a more realistic framework for reproducing clinically relevant conditions for studying pleomorphic liposarcoma. MethodsTrp53fl/fl/Rb1fl/fl/Ptenfl/fl (RPP) mice were used to reliably generate an immunocompetent model of mouse pleomorphic liposarcoma through Cre-mediated conditional silencing of the Trp53, Rb1, and Pten tumor suppressor genes. Evaluation of tumor-infiltrating lymphocytes was assessed with immunostaining for CD4, CD8, and PD-L1, and flow cytometry with analysis of CD45, CD3, CD4, CD8, CD19, F4/80, CD11b, and NKp46 sub-populations. ResultsMice reliably produced noticeable soft-tissue tumors in approximately 6 weeks with rapid tumor growth between 100 and 150 days of life, after which mice reached euthanasia criteria. Histologic features were consistent with pleomorphic liposarcoma, including widespread pleomorphic lipoblasts. Immunoprofiling and assessment of tumor-infiltrating lymphocytes was consistent with other soft-tissue sarcomas. ConclusionGenetically engineered RPP mice reliably produced soft-tissue tumors consistent with pleomorphic liposarcoma, which immunological findings similar to other soft-tissue sarcomas. This model may demonstrate utility in testing treatments for this rare disease, including immunomodulatory therapies.

Paediatric Clear Cell Sarcoma of Scalp Masquerading as Benign Sebaceous Cyst: A Diagnostic Dilemma

Clear Cell Sarcoma of Soft Tissue (CCSST) is a rare and aggressive tumour comprising 1% of all soft tissue sarcomas. They tend to recur locally and have a high predilection for regional, as well as, distant metastases. These tumours usually occur in a young age group; however, their presentation in childhood is extremely rare. They are also known as Malignant Melanoma (MM) of soft parts due to similar histological and Immunohistochemical (IHC) features with MM. Distinguishing between them is very difficult morphologically. Both exhibit high lymph node metastases and positive staining for S-100 protein, Melanoma antigen (Melan-A) and Human Melanoma Black-45 (HMB-45). Here, the authors reported an unusual case of an 11-year-old male child who presented with a slowly progressing scalp swelling that was misdiagnosed as a sebaceous cyst radiologically and on Fine Needle Aspiration Cytology (FNAC), which later turned out to be CCSST on histopathology. Unfortunately, the patient was lost to follow-up, but later presented with multiple cutaneous swellings, emphasising the tumour’s aggressive nature. The index case is being reported as an exception to its age of occurrence and unusual presentation that posed a diagnostic dilemma, leading to a delay in treatment. The treatment for CCSST involves wide surgical resection with negative margins, and adjuvant therapy may be considered based on resection outcomes. In conclusion, the present case underscores the diagnostic challenges posed by CCSST, particularly when it occurs in atypical age groups and locations. Timely recognition and differentiation from similar entities, such as MM, are crucial for appropriate management and improved outcomes.

Ossifying fasciitis: A rare phenomenon and a mimicker for malignancy

Abstract Introduction/Objective Ossifying fasciitis is a relatively rare variant of nodular fasciitis. However, it can mimic malignant lesions, particularly extraosseous osteosarcoma. The purpose of this report is to describe a florid case of ossifying fasciitis with an unusual clinical course in a 17-year-old male. Methods/Case Report A 17-year-old male underwent surgery at an outside hospital for a soft tissue mass in his left upper thigh that had been present for over two years. Pathology was consistent with a benign myofibroblastic tumor. No bone formation was present in the biopsy. Next generation sequencing showed a COL1A1::USP6 gene fusion. The patient was then referred to our institution due to rapid recurrence and unresolving infection at the surgery site. An ultrasound revealed a mass measuring 3.3 x 2.7 x 1.3 cm at the surgery site. The patient subsequently underwent wide surgical resection of the mass.Gross inspection and sectioning of the specimen revealed a well-defined, encapsulated firm calcified lesion with approximately 30% soft pink gelatinous cut surfaces. Microscopically, the lesion was composed of spindle-shaped fibroblastic cells and extensive osteoblastic activity with a zonated transition from immature osteoid to immature woven bone. The diagnosis of ossifying fasciitis was rendered. Results (if a Case Study enter NA) NA Conclusion Rapidly growing lesions can be misinterpreted as sarcomas. Careful microscopic examination and correlation with clinical and radiologic data is critical for accurate diagnosis. Molecular data can be helpful in difficult cases.

Locally aggressive giant basal cell carcinoma of the head with focus on surgical treatment - a case report

Introduction: Basal cell carcinoma is the most common non melanoma skin cancer. It accounts for approximately 80% of all skin cancers. Case report: We presented a 62-year-old patient with a giant, deeply infiltrative, destructive lesion of the head which lasted for 15 years. Microbiological analysis showed contamination, computed tomography (CT) scan showed deep infiltration and bone destruction. The risks of operative treatment were numerous. The treatment was more complicated by infection, infiltrative spreading and the patient's comorbidity. Regardless, we decided on a wide surgical resection with pathohistological evaluation of the resection margins. The patient denied oncological treatment. After 10 years, there were no recurrent tumours. Conclusion: Aggressive surgical treatment is the treatment of choice for giant basal cell carcinoma. In the case of giant locally aggressive and advanced neoplasms, when surgery is not appropriate or not possible, medical treatment becomes oncological.

Abstract 14737: A Case of an Intracardiac Sarcoma Arising From Metastatic Spread of a Primary Phyllodes Tumor of the Breast

Background: Malignant phyllodes tumors with cardiac involvement are rare, presenting diagnostic and management challenges. The benefits of medical and surgical therapies remain unclear. Large-bore aspiration devices are currently only approved for retrieval of venous thromboemboli but may present a valid option for diagnosis and treatment of intracardiac masses in high risk patients. Case Summary: We present the case of a 57-year-old female with a malignant phyllodes tumor of the breast and bilateral pulmonary emboli who presented with an incidental complex intracardiac mass on transthoracic echocardiography (1). A follow-up transesophageal echocardiogram demonstrated masses within the superior vena cava, right atrium, and right ventricle (2a-2b). Aspiration thrombectomy was performed given clinical stability and concern for thrombi with high risk of worsening clot burden. A large-bore aspiration device was used to retrieve the masses in multiple passes (3), which were pathologically consistent with spindle cell sarcoma. A cardiac MRI was completed following aspiration, and multiple mobile masses persisted in the right ventricle (4). The patient was discharged on day nine of hospitalization with therapeutic chemotherapy planned. She had one session of chemotherapy before a fatal cardiac arrest from unknown cause three months after discharge. Discussion: Phyllodes tumors rarely metastasize to the heart, and effective modalities for their diagnosis and management are unclear. Treatment strategies include wide surgical resection of the primary tumor, however mortality remains high in the setting of metastatic spread. Current guidelines recommend following principles of soft tissue sarcomas with chemotherapy. Large-bore aspiration devices may be an effective way to resect intracardiac tumors for both diagnostic and therapeutic purposes as an alternative to invasive surgical intervention for high-risk patients.

Surgical experience in cardiac myxomas at a tertiary hospital

IntroductionCardiac myxomas account for 50% of all benign cardiac tumors. Their clinical presentation varies from embolisms to fever. Our objective was to describe the surgical experience in the resection of cardiac myxomas during an 8-year period. MethodsThis is a retrospective, descriptive study of a series of cases with cardiac myxomas diagnosed from 2014 to 2022 at a tertiary care center. Descriptive statistics were used to define the populational and surgical characteristics. We used Pearson’s correlation to study the relationship between postoperative complications and age, tumor size and affected cardiac chamber. Results31 patients were included, with a predominance of females (1:2 ratio). The prevalence was 0.44%, which was calculated based on the number of cardiac surgeries performed in our unit over the 8-year period. The main clinical manifestation was dyspnea (85%, n = 23), followed by cerebrovascular event (CVE) (18%, n = 5). Atriotomy and resection of the pedicle were performed with preservation of the interatrial septum. Mortality was 3.2%. The postoperative evolution was uneventful in 77%. Tumor recurrence occurred in 2 patients (7%), both debuting with embolic phenomena. No association was observed between postoperative complications or recurrence and tumor size, nor aortic clamping and extracorporeal circulation times with regard to age. ConclusionsFour atrial myxoma resections are performed in our unit per year, with an estimated prevalence of 0.44%. The tumor characteristics described coincide with the previous literature. A relationship between embolisms and recurrences cannot be ruled out. Wide surgical resection of the pedicle and base of tumor implantation may influence tumor recurrence, although further studies are needed.

Current understanding of angiosarcoma: disease biology and evolving treatment.

Angiosarcoma is a very rare soft tissue sarcoma that originates from endothelial cells and typically has a poor prognosis. It is most commonly found in elderly white men and can occur anywhere in the body, particularly in the head, neck, and scalp. Patients who have undergone previous radiation treatment or who have chronic lymphedema also face an elevated risk of this condition. Various genetic changes are suspected to contribute to the development of angiosarcoma, and these changes have been identified as potential targets for treatment. For localized disease, wide surgical resection is often the prudent course of action. A multidisciplinary approach, which may include surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes. In this review, we discuss the general understanding of angiosarcoma and its management, with a particular focus on the current evolving treatments for the disease.

Huge Malignant Peripheral Nerve Sheath Tumors Originating In Neurofibromatosis Type 1

Malignant peripheral nerve sheath tumors (MPNST) are malignant soft tissue neoplasms which account for 2% of all soft tissue sarcomas. These tumors are associated with poor prognosis, propensity to metastasize and high recurrence rates. About half of the MPNST arise from pre-existing neurofibroma and are associated with Neurofibromatosis type I (NF1), 10% are radiotherapy induced others are sporadic. Pathogenesis of MPNST is not fully understood yet. MPNST are common in extremities followed by trunk but are less common in head and neck area. MPNST show limited sensitivity to radiotherapy and chemotherapy and wide surgical resection is mainstay of treatment. We present a series of 3 cases of MPNST of head and neck region originating in patients having NF1.

Surgical experience in cardiac myxomas at a tertiary hospital

IntroductionCardiac myxomas account for 50% of all benign cardiac tumors. Their clinical presentation varies from embolisms to fever. Our objective was to describe the surgical experience in the resection of cardiac myxomas during an 8-year period. MethodsThis is a retrospective, descriptive study of a series of cases with cardiac myxomas diagnosed from 2014 to 2022 at a tertiary care center. Descriptive statistics were used to define the populational and surgical characteristics. We used Pearson’s correlation to study the relationship between postoperative complications and age, tumor size and affected cardiac chamber. Results31 patients were included, with a predominance of females (1:2 ratio). The prevalence was 0.44%, which was calculated based on the number of cardiac surgeries performed in our unit over the 8-year period. The main clinical manifestation was dyspnea (85%, n = 23), followed by cerebrovascular event (CVE) (18%, n = 5). Atriotomy and resection of the pedicle were performed with preservation of the interatrial septum. Mortality was 3.2%. The postoperative evolution was uneventful in 77%. Tumor recurrence occurred in 2 patients (7%), both debuting with embolic phenomena. No association was observed between postoperative complications or recurrence and tumor size, nor aortic clamping and extracorporeal circulation times with regard to age. ConclusionsFour atrial myxoma resections are performed in our unit per year, with an estimated prevalence of 0.44%. The tumor characteristics described coincide with the previous literature. A relationship between embolisms and recurrences cannot be ruled out. Wide surgical resection of the pedicle and base of tumor implantation may influence tumor recurrence, although further studies are needed.

Extra Compartmental Chordoma of Mobile Spine (L3) in a 71-year-old Male: A Case Report and Review of the Literature

Abstract Chordomas of the mobile spine are rare compared to its occurrence in the sacrococcygeal and Clival regions. The treatment modalities include wide surgical resection with or without postsurgery radiation/proton beam therapy. Here, we report a rare case of extra compartmental chordoma of the mobile spine (L3 vertebra) in a 71-year-old male. The patient presented with intractable back and leg pain and difficulty in walking. He was evaluated and managed surgically with complete piecemeal resection through simultaneous posterior and retroperitoneal approaches. He was further managed with radiotherapy after the surgery. The patient is leading a pain-free, independent life postoperatively. This case is being reported for its rarity, complexity of management, and the results. Chordoma of the mobile spine can be managed surgically with total removal of the tumor. Since the whole compartment cannot be resected surgically in the spine (involvement of epidural space and psoas muscle in this case), piecemeal removal with adjuvant therapy is the treatment of choice. The quality of life of the patient is significantly improved after the treatment.

Primary Extrathoracic Extraskeletal Osteosarcoma of the Chest Wall: A Rare Entity

Extraskeletal osteosarcoma (ESOS) is a rapidly growing malignant neoplasm-producing osteoid, located in the soft tissues other than bones or periosteum. Primary ESOS presenting in extrathoracic locations is very rare. The optimal treatment of ESOS is largely unknown. The most common location is the soft tissues of the thigh (46%), upper extremity (20%), intrathoracic (rare), and retroperitoneum (17%). These tumors are usually large while diagnosis, typically measures 10 cm in diameter. The diagnosis is by core biopsy and microscopy sections reveal spindle cells with high NC ratio, myxoid stroma, osteoid matrix, and necrosis. Immunohistochemistry reveals that the tumor cells are positive for Desmin (40%), CD99 (25%), NKX2.2 (30%), and myogenin (15%). The radiological differentials include posttraumatic myositis ossificans. The treatment consists of wide surgical resection and reconstruction depends on the defect. Patients with localized disease were treated with an osteosarcoma-type adjuvant chemotherapy regimen consisting of doxorubicin, and cisplatin. The prognosis is poor when compared with intra-osseous osteosarcoma. More than 90% of patients develop local and distant metastasis and recurrence is between 2 months and 10 years. We hereby describe the case of giant ESOS of the chest wall, which was managed successfully at our center. A 58-year-old male presented with rapid progressive malignant ESOS in the right side chest 20 cm × 20 cm. He was managed by a multidisciplinary team of oncosurgery, plastic surgeon, and onco medicine by neoadjuvant chemotherapy and wide local excision with latissimus dorsi pedicle reconstruction. On a 3-year follow-up, he had no recurrence and doing well.

Oncoplastic and reconstructive breast surgery.

This article provides an overview of the principles and techniques of oncoplastic and reconstructive breast surgery for patients with early-stage breast cancer. Oncoplastic breast surgery (OPBS) with partial breast reconstruction is a natural evolution in the application of breast conserving surgery and permits wide surgical resection of tumours that might otherwise mandate mastectomy and whole breast reconstruction. These reconstructive techniques must be optimally selected and integrated with ablative breast surgery together with non-surgical treatments such as radiotherapy and chemotherapy that may be variably sequenced with each other. A multidisciplinary approach with shared decision-making is essential to ensure optimal clinical and patient-reported outcomes that address oncological, aesthetic, functional and psychosocial domains. Future practice of OPBS must incorporate routine audit and comprehensive evaluation of outcomes.

Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report

BackgroundComposite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis.Case presentationWe present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years.ConclusionsComposite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.

Descripción de 4 casos: tumor desmoide de la mama, diagnóstico y manejo de un tumor infrecuente

IntroductionThe desmoid tumor (DT) of the breast is a rare lesion consisting of proliferation of fibroblastic cells. It can be located intra- or extra-abdominally and the mammary location is exceptional. Although it is a benign lesion, it has a locally aggressive behavior without lymph node or distant metastasis. Its etiology is unknown and 10–20% of cases are related to familial adenomatous polyposis. It presents as a new, painless solid nodule. The treatment has traditionally been surgical resection, with high rates of recurrence despite free margins. Other therapies are being studied. Our objective is to review the management of DT of the breast through the analysis of the casuistry in our center in the last 10 years and the review of the available evidence. MethodsRetrospective descriptive analysis. The cases were identified using the hospital information exploitation system. A narrative review of the literature was performed. ResultsOur series consists of 4 cases, women between 36 and 66 years old. Three of them consulted for a palpable nodule. The CNB presented findings compatible with DT. The cases were presented to a multidisciplinary committee and a wide surgical resection was proposed. The AP confirmed the diagnosis and in one of the cases reported affected margins. During follow-up, recurrence was observed in this case. ConclusionsDTs are infrequent tumors, with clinical repercussions and variable response to treatment. This must include the expectant option and, in any case, be individualized and agreed upon within a multidisciplinary perspective.

Odontogenic myxoma of the mandible: a case report.

We present the unusual case of an odontogenic myxoma involving a 37-year-old female patient, which had acquired large dimensions and involved the right half of the mandible, including the ramus; the patient was treated with large resection surgery, with satisfying medium-term results. Early diagnosis of such lesions is very important, as the patient avoids extensive surgical procedures that involve losing a large part of the jawbones and their subsequent impact on the patient's quality of life. Although there is no fixed treatment plan for the management of odontogenic myxoma, treatment includes surgical management that may range from simple enucleation and curettage to surgical excision; wide surgical resection is appropriate for cases of large size to avoid recurrence.