Vulvar sarcoma outcomes by histologic subtype: a Surveillance, Epidemiology, and End Results (SEER) database review.

OBJECTIVE: Vulvar cancers account for 5% of all gynecologic malignancies; only 1%-3% of those vulvar cancers are primary vulvar sarcomas. Given the rarity of vulvar sarcomas, outcome data specific to histopathologic subtypes are sparse. The aim of this study was to identify clinical and pathologic factors of primary vulvar sarcomas that are associated with survival and may inform treatment decisions. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database was searched for women diagnosed with vulvar sarcoma between 1973 and 2018. We identified 315 patients and reviewed their demographic, clinicopathologic, surgical, and survival information. Statistical analyses included χ2 and t-tests, Kaplan-Meier survival, and Cox regression analyses. RESULTS: The most common histopathologies of vulvar sarcomas were dermatofibrosarcomas (85/315, 27%) and leiomyosarcomas (72/315, 22.9%). Rhabdomyosarcomas (18/315, 5.7%), liposarcomas (16/315, 5.1%), and malignant fibrous histiocytomas (16/315, 5.1%) were less frequent. The majority of patients underwent surgery (292/315, 92.7%), which included lymph node dissections in 21.6% (63/292). Survival and lymph node involvement varied significantly with histologic subtype. The 5-year disease-specific survival for dermatofibrosarcomas, liposarcomas, and fibrosarcomas was 100% and only 60.3% and 62.5% for malignant fibrous histiocytomas and rhabdomyosarcomas, respectively. None of the patients with (dermato)fibrosarcomas, liposarcomas, or leiomyosarcomas had positive lymph nodes, in contrast to rhabdomyosarcomas and malignant fibrous histiocytomas with 77.8% and 40% positive lymph nodes, respectively. The 5-year disease-specific survival for women with positive lymph nodes was 0%. CONCLUSIONS: Vulvar sarcomas are heterogeneous with survival highly dependent on the histopathologic subtype. While surgical excision is the mainstay of treatment for all vulvar sarcomas, staging lymphadenectomy should be deferred for (dermato)fibrosarcomas, liposarcomas, and leiomyosarcomas as there were no cases of lymph nodes metastases.

Undifferentiated Pleomorphic Sarcoma Metastatic to the Orbit.

Undifferentiated pleomorphic sarcoma is a malignancy of mesenchymal origin, which was previously known as malignant fibrous histiocytoma. It is known to occur on rare occasion as a primary orbital tumor, but no known cases of metastatic orbital involvement have been reported since 2002, when the reclassification of these tumors took place. The authors report a patient who presented with a metastasis to the left orbit 2 years after undergoing treatment of a high-grade undifferentiated pleomorphic sarcoma of the right thigh. Histopathology of the orbital mass was similar to the primary tumor biopsy prior to neoadjuvant chemotherapy and radiation. The appearance was markedly altered in the subsequent excisional tissue, which showed treatment changes. Immunohistochemistry and genetic testing also supported the metastatic nature of the orbital lesion. The patient's tumor progressed rapidly despite systemic targeted therapy and orbital exenteration was performed. At 2 years follow up, the patient remained without evidence of tumor recurrence in the socket.

En bloc resection and prosthesis implantation to treat malignant fibrous histiocytoma of the humerus.

BACKGROUND: Malignant fibrous histiocytoma (MFH) of the bone is a rare tumor. Most studies comparing limb salvage and amputation have reported that limb salvage had no adverse effect on the long-term survival of patients. This study evaluates the oncological outcomes of limb salvage procedures that were used for 15 patients with MFH of the humerus. OBJECTIVES: The aim of this study was to assess the functional and oncological outcomes of patients with malignant fibrous histiocytoma of the humerus after en bloc resection and prosthesis implantation. MATERIAL AND METHODS: A retrospective review of the charts of 15 patients who had undergone resection of malignant fibrous histiocytoma of the humerus followed by reconstruction with prosthesis was used in this study. A functional evaluation was based on Enneking's modified system of the functional evaluation of surgical management for musculoskeletal tumors. Complications of the procedures were also analyzed. RESULTS: Eight men and 7 women at an average age of 52.9 years were included in the study. The tumor involved the distal humerus in 3 patients, the proximal humerus in 8 patients and the mid-shaft humerus in 4 patients. Excellent results were achieved in 4 patients, good to fair in 10 and poor in 1. One patient had local recurrence. Pulmonary metastases occurred in 6 patients. CONCLUSIONS: Limb salvage surgery with chemotherapy is a viable treatment option for patients with malignant fibrous histiocytoma of the humerus.

Primary angiomatoid fibrous histiocytoma of the lung with mediastinal lymph node metastasis.

Angiomatoid fibrous histiocytoma (AFH), a rare soft tissue tumor of borderline malignancy, typically occurs in the subcutis of the accessible soft tissues, most often in children and young adults. Lymph node and/or distant metastasis is seen in less than 2% of patients with AFH. Exceptionally rare examples of AFH have been reported in visceral locations, including the lung. We report a genetically confirmed primary pulmonary AFH in a 70-year-old woman with mediastinal lymph node metastasis, representing to the best of our knowledge the first report of metastases from a visceral AFH.

Retrospective characterisation of solitary cutaneous histiocytoma with lymph node metastasis in eight dogs.

OBJECTIVES: To describe a small subset of canine solitary cutaneous histiocytoma in which lymph node metastasis has been documented. METHODS: Cases of dogs with solitary cutaneous histiocytoma lesions and regional lymph node metastasis diagnosed via histopathology were found through a retrospective search of the databases of IDEXX Laboratories and the University of California, Davis Veterinary Medical Teaching Hospital Clinical Diagnostic Laboratories. Information on signalment, history and clinical follow-up was obtained from the submittal form and/or via a questionnaire to the submitting veterinarian. Slides were available for review in seven cases and when possible immunohistochemistry was reviewed or performed by a single pathologist. RESULTS: Eight cases met the inclusion criteria. The neoplasms had the typical appearance of histiocytomas. All tested samples were immunoreactive for CD18 and lacked immunoreactivity for other lymphocyte markers and CD11d. Immunoreactivity for E-cadherin varied among the neoplasms tested. Outcome was known for five dogs and at the time of manuscript preparation three of those dogs were alive 1682 days, 570 days and 318 days post-diagnosis. Of the other two dogs with known outcome, one was euthanased shortly after diagnosis and another was hit by a car. Of the dogs that were eventually lost to follow-up, one was reported to be disease-free 1003 days after diagnosis. CLINICAL SIGNIFICANCE: Metastatic histiocytoma is rarely reported and distinction from aggressive disease processes such as histiocytic sarcoma may be difficult. Based upon a small number of cases with known outcomes, some dogs with solitary metastatic histiocytoma may experience favourable outcomes.

DNA copy number changes in tumors within the spectrum of cellular, atypical, and metastasizing fibrous histiocytoma.

BACKGROUND: Cutaneous fibrous histiocytoma (FH) is a common mesenchymal neoplasm. Metastasis is rare, disproportionately occurring among the aneurysmal, cellular, atypical, and deep variants. OBJECTIVE: We determined whether DNA copy number changes occurred in atypical FH (AFH), and whether they were similar to those in metastasizing FH (MetFH) and benign cellular FH (CFH). METHODS: Five primary tumors of MetFH were evaluated by array-based comparative genomic hybridization analysis, with tissue from local recurrences and lung metastases in 2 and 2 patients, respectively. Seven indolent AFH and 5 CFH were identified for comparison. RESULTS: Substantial differences between the groups were found both in the frequency of chromosomal aberrations (higher among MetFH and absent or solitary in CFH) and array-based comparative genomic hybridization profiles (frequent gains of 7 and 8q and losses of Xq in MetFH; recurrent losses of chromosomes 9 and 22 in AFH; isolated loss of 5q and gain in chromosome 20 in 2 CFH). Fatal MetFH cases (2 of 5 cases) exhibited the highest rate of chromosomal aberrations. LIMITATIONS: This study included a small sample size with a short-term follow-up. CONCLUSIONS: Benign CFH, indolent AFH, and MetFH represent distinct biological entities within the spectrum of FH; array-based comparative genomic hybridization may be a tool in recognizing FH cases with metastatic potential and increasingly aggressive behavior.

Multimodality management of metastatic patients with soft tissue sarcomas may prolong survival.

OBJECTIVES: Patients who develop metastatic disease from soft tissue sarcoma have a poor prognosis. The purpose of this study was to identify metastatic survival rates and identify prognostic variables that predict for these outcomes. METHODS: Between 2000 and 2010, 182 patients with stage I to IV primary soft tissue sarcomas of the extremity and trunk were treated with multimodality treatment. Fifty-five patients developed or presented with metastasis. We retrospectively analyzed prognostic factors for metastatic survival. Metastatic survival between groups was compared with the log-rank test. Survival curves were estimated by Kaplan-Meier plots. Multivariate analysis was performed using the Cox proportional hazards model. RESULTS: Median follow-up was 3.1 years. Median metastatic survival was 24.2 months. Median metastatic survival in those undergoing multimodality therapies was 40 versus 22 months in those receiving single modality treatments. In single predictor Cox models, age, stage, number of lung metastases, location of metastases, and primary disease were significant for metastatic survival. On multivariate analysis, number of pulmonary metastases, histology, stage, and location of primary disease predicted for metastatic survival. Patients who had pulmonary-only disease had improved metastatic survival versus those that had extrapulmonary with or without pulmonary metastatic disease (38 vs. 15 mo). Patients who had ≤5 pulmonary metastasis had improved metastatic survival versus those that had >5 pulmonary lesions (55 vs. 22 mo). CONCLUSIONS: This analysis shows that >5 pulmonary metastasis, malignant fibrous histiocytoma histology, stage III disease, and proximal lower extremity sarcomas are associated with decreased metastatic survival. Moreover, aggressive multimodality management of metastatic disease may prolong metastatic survival.

Survival impact of pulmonary metastasectomy for patients with head and neck cancer.

BACKGROUND: The purpose of this study was to evaluate the survival impact and prognostic factors of pulmonary metastasectomy in patients with pulmonary metastasis from head and neck cancer. METHODS: A retrospective study of 69 patients was analyzed. Twenty-four patients (35%) underwent pulmonary metastasectomy, and the remaining 45 patients (65%) were treated with chemotherapy or best supportive care. RESULTS: The 1-year overall survival (OS) of 69 patients was 28%. Pulmonary metastasectomy (p = .01) and histology (p < .001) had a significant impact on the prognosis. One-year OS of patients who underwent metastasectomy and those who did not was 90% and 35%, respectively. In the metastasectomy group, recurrence of primary ahead of lung metastasis (p = .006) and disease-free interval (DFI; ≤21.4 months; p = .046) were significant negative prognostic factors. CONCLUSION: Pulmonary metastasectomy has an impact on survival in carefully selected patients, especially for those with a long DFI and with no recurrence of primary cancer ahead of lung metastasis.

Laparoscopic approach for isolated splenic metastasis: comprehensive literature review and report of 6 cases.

BACKGROUND: Splenic metastases are extremely unusual. The aim of this paper is to review the world experience to date concerning to laparoscopic approach for splenic metastasis, and to report ours at the Hospital de Sant Pau. METHODS: Literature review was carried out and relevant reports on laparoscopic approach to splenic metastasis were obtained. In addition, we reviewed our patient's database and retrieved the data of those who underwent laparoscopic splenectomy due to splenic involvement. RESULTS: Electronic search yielded 8 relevant articles on open splenectomy and 7 articles on laparoscopic splenectomy for splenic metastasis. During the study period, 6 patients were operated on laparoscopically. Primary neoplasms were 3 melanomas, 2 colon carcinomas, and 1 malignant fibrous histiocytoma. None of the patients had surgical complications. Outcome ranged from 2-month to 11-year survival. CONCLUSIONS: The laparoscopic approach for splenic metastasis is feasible. Of course, it must be individualized in each case.

A case of cutaneous malignant fibrous histiocytoma with multiple organ metastases.

Malignant fibrous histiocytoma is a soft tissue sarcoma that most commonly occurs in the extremities and rarely metastasizes cutaneously. A 79-year-old male patient consulted a dermatologist 11 months after recognizing an intractable ulcer on the right mandible. Punch biopsy revealed eosinophilic tumor cells in the dermal area and proliferation of rich spindle cells. Malignant fibrous histiocytoma or atypical fibroxanthoma was suspected and he was referred to our hospital. Red plaque tumors on the right mandible and right temple were 30 mm and 15 mm in size, respectively. The right mandible lesion was ulcerated. Immunohistochemically, the lesions were positive for CD10, CD74 and alpha-smooth muscle actin. Radiological analysis revealed multiple organ metastases, including bone, liver, lung and skin on the right temple. The patient was diagnosed with malignant fibrous histiocytoma, stage IV and died 8 weeks after the first visit due to respiratory failure. Cutaneous malignant fibrous histiocytoma has a poor prognosis resulting in death.

Electron microscopy in the diagnosis of malignant fibrous histiocytoma of the lung presenting as metastasis to the maxillary gingiva.

A rare case of an initial presentation of a metastatic lesion on the gingiva, arising from malignant fibrous histiocytoma (MFH) of the lung is reported. The role of electron microscopy in the diagnosis of soft tissue sarcomas has been studied. The ultrastructural details of a MFH are described and illustrated. The patient died within 9 weeks of the initial presentation with a gingival mass as is normal in these cases. He developed metastatic disease of the liver, lymph nodes, and bone. The role of electron microscopy in the diagnosis of soft tissue sarcomas is discussed.

Renal pleomorphic undifferentiated sarcoma: a rarity.

A 70-year-old male presented with progressive weight loss for eight months. Radiological imaging showed a large tumor in the right kidney. The patient underwent right open radical nephrectomy and histopathology revealed pleomorphic undifferentiated sarcoma (PUS) earlier known as malignant fibrous histiocytoma (MFH). One year after surgery, the patient developed pulmonary metastasis. Unfortunately, the patient died after six months.

The evolving conception and management challenges of malignant fibrous histiocytoma.

BACKGROUND: Malignant fibrous histiocytoma (MFH) is a rare and aggressive tumor. Mohs micrographic surgery (MMS) has been reported as an effective treatment, although most cases were published before advances in cytopathologic techniques led to reclassification of many tumors. OBJECTIVE: To evaluate a contemporary cohort of individuals with MFH and analyze management practices. METHODS: We reviewed all cases of MFH diagnosed at our institution from January 1995 to December 2010, evaluating 839 records to identify 36 patients undergoing management of tumors of the head and neck. RESULTS: Seventeen of the total 36 patients (47%; mean age 67) experienced tumor recurrence, and 10 (28%) developed metastases. Seven of nine patients initially treated with MMS (78%), and 10 of 24 (42%) treated with WLE experienced recurrence (p = .06). Patients treated with MMS had smaller tissue defects after surgery. The mean contemporary recurrence rate of MFH treated with MMS is significantly higher (58.8%) than the cumulative recurrence rate reported before 2000 (7.4%) (p < .001). CONCLUSIONS: Our study is consistent with reports of MFH as an aggressive neoplasm and describes the largest population treated with MMS in 3 decades. The changing conception of MFH, along with a propensity for in-transit metastases, may explain higher contemporary recurrence rates.

Malignant fibrous histiocytoma of the ovary: a case report.

Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma of late adult life occurring predominantly in the extremities and the retroperitoneum. MFH of the ovary is very rare, with only six cases previously reported. A 67-year-old woman with a right pelvic tumor highly suspicious of ovarian carcinoma was submitted to exploratory laparotomy. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, total omentectomy, pelvic and paraaortic lymphadenectomy with right hemicolectomy along with permanent cutaneous ileostomy were performed. Since a storiform-pleomorphic type of MHF was diagnosed from histopathological and immunohistochemical findings, chemotherapy was proposed as the postoperative treatment. Despite extensive surgery with negative surgical margins, the patient had recurrence of the tumor within four months, and was submitted to secondary surgery. A combination of chemo- and radiotherapy was performed postoperatively, but the patient developed respiratory problems and died one year later from the primary diagnosis.

Rapidly progressive descending aortic pseudoaneurysm resulting from primary malignant fibrous histiocytoma.

A 66-year-old man with a history of asymptomatic bilateral swelling of the axillary lymph nodes was admitted with hoarseness and pain of the right knee. Computed tomography showed a large pseudoaneurysm deriving from descending aorta. We planned to resect the pseudoaneurysm, and repair the aortic wall. Resection of the pseudoaneurysm and patch-closure of the defect of the aorta were performed successfully. Pathological examination of the pseudoaneurysm revealed malignant fibrous histiocytoma. The clinical course was very miserable. Intrathoracic recurrence occurred 7 months after surgery, and he died of multiple brain metastases 10 months postoperatively.

"Malignant" mitral stenosis.

Symptomatic mitral stenosis caused by a left atrial mass as the first sign of metastasis of a malignant tumor is extremely rare and frequently associated with poor prognosis. We report a case of a 59-year-old man with a history of grade 3 malignant fibrous histiocytoma on his left tigh treated by limb-sparing surgery 17 months earlier, who was admitted with 10-days of worsening dyspnea. Imaging revealed a left atrial mass protruding through the mitral valve that resulted in severe mitral stenosis. Biopsy confirmed metastasis of malignant fibrous histiocytoma.

Unusual hematogenous brain metastasis in malignant fibrous histiocytoma of the maxillary sinus.

Malignant fibrous histocytoma (MFH) of the maxillary sinus is believed to be a rare form of soft tissue sarcoma with a low frequency of distant metastasis. In this study, we provide a histological documentation of the hematogenous spread of MFH to the brain and report a case of maxillary sinus MFH with unusual metastasis to the brain. To our knowledge, this is the first case of a direct histological diagnosis of maxillary sinus MFH with brain metastasis via a hematogenous route.