Malignant Peripheral Nerve Tumors Research Articles

Immunotherapeutic treatments for spinal and peripheral nerve tumors: a primer.

Spinal and peripheral nerve tumors are a heterogeneous group of neoplasms that can be associated with significant morbidity and mortality despite the current standard of care. Immunotherapy is an emerging therapeutic option to improve the prognoses of these tumors. Therefore, the authors sought to present an updated and unifying review on the use of immunotherapy in treating tumors of the spinal cord and peripheral nerves, including a discussion on mechanism of action, drug delivery, current treatment techniques, and preclinical and clinical studies. Current data in the literature regarding immunotherapy were collated and summarized. Targeted tumors included primary and secondary spinal tumors, as well as peripheral nerve tumors. Four primary modalities of immunotherapy (CAR T cell, monoclonal antibody, viral, and cytokine) have been reported to target spine and peripheral nerve tumors. Of the primary spinal tumors, spinal cord astrocytomas had the most preclinical evidence supporting immunotherapy success with CAR T-cell therapy targeting the H3K27M mutation, whereas spinal schwannomas and ependymomas had the most evidence reported for monoclonal antibody therapy preclinically. Of the secondary spinal tumors, primary CNS lymphomas demonstrated some clinical response to immunotherapy, whereas multiple myeloma and bone tumor experiences with immunotherapy were largely limited to concept only. Within peripheral nerve tumors, the use of immunotherapy to treat neurofibromas in the setting of syndromes has been suggested in theory, and possible immunotherapeutic targets have been identified in malignant peripheral nerve tumors. To date, there have been 2 clinical trials involving spine tumors and 2 clinical trials involving peripheral nerve tumors that have reported results, all of which are promising but require validation. Immunotherapy to treat spinal and peripheral nerve tumors has become an emerging area of research and interest. A large amount of preclinical data supporting the translation of this therapy into practice, aimed at ameliorating the poor prognoses of specific tumors, have been reported. Future clinical studies for translation will focus on the optimal therapy type and administration route to best target these tumors, which often preclude total surgical resection given their proximity to the neural and vascular elements of the spine.

Schwannomatosis of the tibial nerve

<p class="abstract">Schwannoma is the most common type of benign tumor arising from the sheaths of the peripheral nerves. It occurs as a solitary tumor in most cases, but when it appears in multiple forms, it is necessary to differentiate it from plexiform schwannoma, schwannomatosis, neurofibroma and malignant peripheral nerve tumors. The authors experienced schwannomatosis in the tibial nerve without the features of neurofibromatosis type 2, so here we present a case report and literature review.</p>

Comprehensive clinicopathologic characteristics of intraabdominal neurogenic tumors: Single institution experience.

BACKGROUNDNeurogenic tumors are rare but represent an important consideration in the differential diagnosis of abdominal mesenchymal tumors. Reports on their incidence, pathological features and clinical characteristics are scarce. AIMTo advance the overall knowledge on the histologic, immunohistochemical, clinical and radiologic characteristics of neurogenic tumors through this case series.METHODSAn established database of a nationwide tertiary referral center, covering a 15-year period (2005 and 2020), was retrospectively re-evaluated. Diagnoses of neurogenic tumor cases were confirmed by two experts following review of the macroscopic, histological and immunohistochemical records along with findings from analysis of archived tissue sections for each included patient. Tissue microarrays were constructed for cases lacking necessary immunohistochemical studies. Clinical data and follow-up information were collected from the hospital records and the patients themselves, when available.RESULTSThe study included 19 cases of intraabdominal neurogenic tumors, representing 12 women and 7 men, between 18 and 86 years of age (median: 51 years). Final confirmed diagnoses were 12 schwannomas, 2 diffuse submucosal neuro-fibromatoses, 2 ganglioneuromas, 2 malignant peripheral sheath nerve tumors, and 1 mucosal Schwann cell hamartoma. Sizes of the tumors were variable, with a median diameter of 4 cm; the two largest (> 10 cm) were schwannomas. The majority of cases were asymptomatic at presentation, but the most frequent symptom was abdominal pain. Gastrointestinal tract lesions were detected with endoscopy and extra-luminal lesions were detected with cross-sectional imaging. All cases were S100-positive and CD117-negative; most cases were negative for desmin, epithelial membrane antigen, smooth muscle actin and CD34. In all but 5 cases, the Ki67 proliferation index was ≤ 1%.CONCLUSIONRe-evaluation of 19 cases of abdominal neurogenic tumors demonstrated con-siderable variability in clinicopathologic characteristics depending on location, dimension and histological features.

Clinical and pathological features of upper limb nerve tumors - four years retrospective study.

Benign peripheral nerve tumors and malignant peripheral nerve tumors are rarely found in patients with upper limb tumors. A four-year retrospective study was conducted on patients with tumors in the upper limb area admitted to the Emergency Clinical Hospital, Bucharest, Romania. Seventeen patients were admitted within this time range, 15 of which were benign and two malignant. All patients required surgical intervention after thorough clinical and imaging evaluation. Benign masses were removed, follow-up examination revealing no local recurrent, as well as good function recovery. On the other hand, malignant tumors due to their highly aggressive features, both determined local recurrence, one requiring upper limb amputation, the other presenting metastases.

Imaging of non-neurogenic peripheral nerve malignancy-a case series and systematic review.

To evaluate the frequency, clinico-pathologic and imaging features of malignant tumors in peripheral nerves which are of non-neurogenic origin (non-neurogenic peripheral nerve malignancy-PNM). We retrospectively reviewed our pathology database for malignant peripheral nerve tumors from 07/2014-07/2019 and performed a systematic review. Exclusion criteria were malignant peripheral nerve sheath tumor (MPNST). Clinico-pathologic and imaging features, apparent diffusion coefficient (ADCmin), and standard uptake values (SUVmax) are reported. After exclusion of all neurogenic tumors (benign = 196, MPNST = 57), our search yielded 19 non-neurogenic PNMs (7%, n = 19/272), due to primary intraneural malignancy (16%, n = 3/19) and secondary perineural invasion from an adjacent malignancy (16%, n = 3/19) or metastatic disease (63%, n = 12/19). Non-neurogenic PNMs were located in the lumbosacral plexus/sciatic nerves (47%, n = 9/19), brachial plexus (32%, n = 6/19), femoral nerve (5%, n = 1/19), tibial nerve (5%, n = 1/19), ulnar nerve (5%, n = 1/19), and radial nerve (5%, n = 1/19). On MRI (n = 14/19), non-neurogenic PNM tended to be small (< 5cm, n = 10/14), isointense to muscle on T1-W (n = 14/14), hyperintense on T2-WI (n = 12/14), with enhancement (n = 12/12), low ADCmin (0.5-0.7 × 10-3mm2/s), and variable metabolic activity (SUVmax range 2.1-13.1). A target sign was absent (n = 14/14) and fascicular sign was rarely present (n = 3/14). Systematic review revealed 89 cases of non-neurogenic PNM. Non-neurogenic PNMs account for 7% of PNT in our series and occur due to metastases and primary intraneural malignancy. Although non-neurogenic PNMs exhibit a non-specific MRI appearance, they lack typical signs of neurogenic tumors such as the target sign. Quantitative imaging features identified by DWI (low ADC) and F18-FDG PET/CT (high SUV) may be helpful clues to the diagnosis.

P3.16-23 Soft-Tissue Sarcoma of the Chest Wall, Surgical Resection: Analysis Our Cases Period of Time Decembre 2004-July 2017

Soft tissues include muscle, fat, blood vessels, nerves, tendons and joint linings (synovial tissue). Cancerous tumors (sarcomas) of the soft tissue are rare, but there are many types. Because soft tissue sarcomas arise from a number of different kinds of tissue, they can occur anywhere in the body. Outcomes for people with soft tissue sarcomas depend on the type, size, grade, stage and location of the tumor, as well as a person's age and general health. Clasification from grade are :low ,midle ,high diferenciations..Aim of Study: Our objective was to introduce a patient diagnosed with neglectetd giant soft tissue chest wall liposarcoma . Records of 32 patients admitted to our institution from January 2004 to July 2017 were treated in our clinic’ A patient with the initials K.B bored 17/06/1950 .He was admitted to our hospital in thoracal surgery at 19.09.2012 with diagnosis : soft tissue tumor left chest wall .Pacienti without other diseases . We did all necessary examinations , CT , MRI and determined the extent of tissue tumor. Ages ranged from 13 to 86 years (median, 38 years); the ratio of male to female patients was 3:1. The initial complaint was mass or pain in 98% of the cases. Histologic types were as follows: desmoid tumor (n = 5), 15 %; liposarcoma (n = 7), 21%; rhabdomyosarcoma (n = 6), 18%); fibrosarcoma (n =4 , 12%); malignant peripheral nerve tumor (n = 3, 9%); malignant fibrous histiocytoma (n = 1, 3%); tenosynovial sarcoma (n = 1),3 %; hemangiopericytoma (n = 2) ,6%%); alveolar soft part sarcoma (n = 2),6%); and other types(lymphoma) (n = 2),6%). Resection was the primary treatment in 28 cases (87%).All patients were treated by multimodal treatment ,chemoradition therapy and surgery. Local recurrence developed in 25%. Metastases occurred in 15 (46%) of the cases (metachronous in 12, synchronous in 3) and were more common in patients with high-grade disease than in those with low-grade disease Overall 5-year survival was 60%. Five-year survival rate for those with high-grade sarcomas was significantly lower than that for low-grade sarcomas .Tumor size and age of patient were not prognostic. Surgery is important in the treatment of most sarcomas.[Additional treatments, including chemotherapy and radiation therapy, may be administered before and/or after surgery. Thoracic wall soft-tissue sarcomas are best controlled by wide surgical resection.

Peripheral neuroblastoma in an adult: Rare disease at a rare site

Adult peripheral neuroblastoma represents less than 1% of all malignant primary tumors of peripheral nerves. We report a young adult who presented with swelling over the left wrist with left epitrochlear and axillary lymph nodes. Incision biopsy of the swelling was suggestive of malignant small round cell tumor. On immunohistochemistry, cells were positive for synaptophysin, chromogranin and nonspecific enolase and negative for leucocyte common antigen, cytokeratin, CD99 and myogenin. Urinary vanillyl mandelic acid and homovanillic acid levels were elevated. The patient received 8 cycles of chemotherapy (OJEC). Reassessment positron emission tomography-computed tomography scan showed a complete metabolic response at the primary site and partial response at left axillary lymph nodes. The patient underwent axillary lymph node clearance followed by radiotherapy to the tumor bed and lymph node regions. The patient could not afford autologous haematopoetic stem cell transplant and was started on isotretinoin maintenance. He is on follow-up for 12 months and disease free.

Peripheral neuropathy in neurofibromatoses

WCN 2013 No: 2699 Topic: 7 Neuromuscular disorders Peripheral neuropathy in neurofibromatoses M. Poloni, R. Barbo. Neurosciences, HPG 23, Bergamo, Italy; Neurosciences, ICH Gavazzeni, Bergamo, Italy Neurofibromas are benign tumors which originate from peripheral nerves and are, together with others pathognomonic symptoms, a common finding in Neurofibromatosis 1 (NF1) conversely Neurofibromatosis 2 (NF2) or central neurofibromatosis is characterized by abnormal growth of Schwann cell tumors called schwannomas (SCH) at vestibular nerves, beyond meningiomas, ependimomas and SCHs of other cranial nerves or different Central Nervous System (CNS) tumors. NF1 is a genetic disorder transmitted thorough chromosome 17 due to neurofibromin alterations and abnormal tumors growth with rare Malignant Peripheral Nerve Tumors (MPNT), while NF2 is linked to merlin abnormalities and is at same genetically transmitted but through chromosome 22. Peripheral neuropathy (PN) appears rarely in NF1, ranging from 0 to 4.3 % of patients in different casistics, while retains only instrumental evidence in NF2 where mononeuropathy, generally as a foot drop or a facial palsy, is relatively common. Three interesting cases of important and distressing peripheral neuropathy, one affected by NF1 and two brothers affected by NF2, have been seen and studied at the NF Ambulatorial Center of Bergamo: we want to share the clinical and instrumental characteristics of these patients, discussing the differential diagnosis and arguing the different pathogenetic mechanisms. doi:10.1016/j.jns.2013.07.1666 Abstract WCN 2013 No: 2686 Topic: 7 Neuromuscular disorders Facial onset sensory and motor neuronopathy: A neurodegenerative TDP-43 proteinopathy? WCN 2013 No: 2686 Topic: 7 Neuromuscular disorders Facial onset sensory and motor neuronopathy: A neurodegenerative TDP-43 proteinopathy? E.P. Bosch, B.P. Goodman, J.A. Tracy, P.J.B. Dyck, C. Giannini. Neurology, Mayo Clinic Arizona, Scottsdale, AZ, USA; Neurology, Mayo Clinic Rochester, Rochester, MN, USA; Pathology, Mayo Clinic Rochester,

Anatomic MR Imaging and Functional Diffusion Tensor Imaging of Peripheral Nerve Tumors and Tumorlike Conditions

A number of benign and malignant peripheral nerve tumor and tumorlike conditions produce similar imaging features on conventional anatomic MR imaging. Functional MR imaging using DTI can increment the diagnostic performance in differentiation of these lesions. Our aim was to evaluate the role of 3T anatomic MR imaging and DTI in the characterization of peripheral nerve tumor and tumorlike conditions. Twenty-nine patients (13 men, 16 women; mean age, 41±18 years; range, 11-83 years) with a nerve tumor or tumorlike condition (25 benign, 5 malignant) underwent 3T MR imaging by using anatomic (n=29), functional diffusion, DWI (n=21), and DTI (n=24) techniques. Images were evaluated for image quality (3-point scale), ADC of the lesion, tractography, and fractional anisotropy of nerves with interobserver reliability in ADC and FA measurements. No significant differences were observed in age (benign, 40±18 versus malignant, 45±19 years) and sex (benign, male/female=12:12 versus malignant, male/female=3:2) (P>.05). All anatomic (29/29, 100%) MR imaging studies received "good" quality; 20/21 (95%) DWI and 21/24 (79%) DTI studies received "good" quality. ADC of benign lesions (1.848±0.40×10(-3) mm2/s) differed from that of malignant lesions (0.900±0.25×10(-3) mm2/s, P<.001) with excellent interobserver reliability (ICC=0.988 [95% CI, 0.976-0.994]). There were no FA or ADC differences between men and women (P>.05). FA of involved nerves was lower than that in contralateral healthy nerves (P<.001) with excellent interobserver reliability (ICC=0.970 [95% CI, 0.946-0.991]). ADC on DTI and DWI was not statistically different (P>.05), with excellent intermethod reliability (ICC=0.943 [95% CI, 0.836-0.980]). Tractography differences were observed in benign and malignant lesions. 3T MR imaging and DTI are valuable methods for anatomic and functional evaluation of peripheral nerve lesions with excellent interobserver reliability. While tractography and low FA provide insight into neural integrity, low diffusivity values indicate malignancy in neural masses.

Hemangiopericytoma-Like Dermatofibroma With Mast Cells

We report an unusual case of hemangiopericytoma-like dermatofibroma in the right shoulder of an 82-year-old patient with a well-defined nodular growth located in the dermis. Microscopic study revealed a band of haphazardly arranged cells with a vascular component of gaping, simple, endothelial-lined vascular structures with intervening postcapillary venules and capillary-sized slit-like "staghorn" vascular channels filled with erythrocytes; abundant mast cells were also observed. The neoplasm cells were positive for CD68 and Factor XIIIA and negative for CD34. Few data have been published on the presence of abundant mast cells (tryptase and CD117 positive) in these neoplasm. The differential diagnosis of this entity should consider other spindle cell neoplasm, including hemangiopericytoma/solitary fibrous tumor, dermatofibrosarcoma protuberans, myopericytoma, angioleiomyoma, amelanotic melanoma, pecoma, and benign and malignant peripheral nerve tumors. We present an infrequent case of dermatofibroma with a vascular pattern resembling hemangiopericytoma and the presence of abundant mast cells, which may be responsible for this vascular component.

Les sarcomes primitifs du médiastin

Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics.Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given.The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died.Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

Nerve Tumors

A 58-year-old right-handed woman was referred for a 5-year history of numbness in the left median nerve distribution and a slowly growing volar wrist mass. Her examination was notable for a nontender, firm, midline mass extending from the proximal wrist flexion crease to the distal palmar crease. Phalen's test and Tinel's tests were positive. Semmes-Weinstein monofilament testing was 3.61 in the median nerve distribution and 2.83 in the remainder of the hand. Electrophysiological tests demonstrated muscle denervation within the thenar muscles and undetectable motor and sensory latencies. Magnetic resonance imaging (MRI) revealed median nerve enlargement extending from the distal forearm to the distal metacarpal region with fatty infiltration. Educational Objectives •State the most common types of peripheral nerve sheath tumors. •Discuss the differences between neurofibromas and schwannomas. •List the schwannoma characteristics associated with permanent neurologic dysfunction following excision via intracapsular enucleation. •Describe risk factors associated with elevated risk for peripheral nerve tumors. •Summarize the MRI findings consistent with a malignant peripheral nerve tumor. Earn up to 2 hours of CME credit per JHS issue when you read the related articles and take the online test. To pay the $20 fee and take this month’s test, visit http://www.assh.org/professionals/jhs. •State the most common types of peripheral nerve sheath tumors. •Discuss the differences between neurofibromas and schwannomas. •List the schwannoma characteristics associated with permanent neurologic dysfunction following excision via intracapsular enucleation. •Describe risk factors associated with elevated risk for peripheral nerve tumors. •Summarize the MRI findings consistent with a malignant peripheral nerve tumor. Earn up to 2 hours of CME credit per JHS issue when you read the related articles and take the online test. To pay the $20 fee and take this month’s test, visit http://www.assh.org/professionals/jhs. Journal CME QuestionsJournal of Hand SurgeryVol. 35Issue 9Preview Full-Text PDF

Tyrosinase Expression in Malignant Melanoma, Desmoplastic Melanoma, and Peripheral Nerve Tumors

Tyrosinase Expression in Malignant Melanoma, Desmoplastic Melanoma, and Peripheral Nerve Tumors

Tyrosinase expression in malignant melanoma, desmoplastic melanoma, and peripheral nerve tumors.

Tyrosinase expression in malignant melanoma, desmoplastic melanoma, and peripheral nerve tumors.

Histopathologic type: an independent prognostic factor in primary soft tissue sarcoma of the extremity?

We attempted to define the effect of tumor histotype on local recurrence, distant metastasis, and disease-specific survival in patients with surgically treated primary extremity sarcoma. A total of 951 patients with primary, localized soft tissue extremity sarcoma were followed up prospectively. Patient- and tumor-related variables, including histopathologic type, were used to identify independent prognostic factors for the study end points of local recurrence, distant recurrence, and disease-specific survival. There were 137 local recurrences, and significant adverse prognostic factors for local recurrence were patient age >50 years, microscopically positive margins, and malignant peripheral nerve tumor. Adverse prognostic factors for distant recurrence (200 patients) were tumor size >5 cm, tumors beneath the investing fascia, high tumor grade, and leiomyosarcoma. Of the 199 patients who died of disease-related causes, patient age >50 years, tumors beneath the investing fascia, high tumor grade, microscopically positive margin, tumor size >5 cm, leiomyosarcoma, and malignant peripheral nerve tumor were adverse prognostic factors. These data suggest that differences in biological behavior may exist between sarcoma histotypes and deserve further study.

Tyrosinase expression in malignant melanoma, desmoplastic melanoma, and peripheral nerve tumors.

Pathologists may encounter problems in the differential diagnosis of malignant melanoma, spindle and epithelioid neoplasms of peripheral nerves, and fibrohistiocytic tumors. Tyrosinase has been demonstrated to be a sensitive marker for melanoma. To determine the specificity of tyrosinase expression in the differential diagnosis of melanoma, desmoplastic melanoma, and peripheral nerve sheath tumors. Immunoreactivity for tyrosinase, HMB-45 (anti-gp100 protein), S100 protein, CD34, and vimentin was studied in 70 tumors, including 15 melanomas (5 desmoplastic, 4 amelanotic, 6 melanotic), 13 malignant peripheral nerve sheath tumors; 10 schwannomas (1 pigmented), 12 neurofibromas (4 pigmented), and 20 fibrohistiocytic tumors (10 dermatofibrosarcoma protuberans and 10 dermatofibromas). Microwave-based antigen retrieval was performed in 10mM citrate buffer, pH 6.0, for 20 minutes at 121 degrees C. All melanomas demonstrated positive immunostaining for tyrosinase, HMB-45, and S100 protein. Immunoreactivity for HMB-45 was generally stronger than that for tyrosinase in amelanotic lesions and significantly stronger in 1 of the desmoplastic lesions. The 4 pigmented neurofibromas were focally positive for tyrosinase, but did not stain for HMB-45. The pigmented schwannoma was focally positive for both tyrosinase and HMB-45. The malignant peripheral nerve sheath tumors, dermatofibrosarcoma protuberans, and dermatofibromas were nonreactive for tyrosinase and HMB-45. Our results support the sensitivity of tyrosinase expression and demonstrate the relative specificity of tyrosinase as a marker for melanocytic lesions, including desmoplastic melanoma, although pigmented peripheral nerve tumors may demonstrate focal positive staining. Immunoreactivity for tyrosinase and HMB-45 may have been enhanced by the microwave-based antigen-retrieval technique used in this study.

Carboplatin/etoposide induces remission of metastasised malignant peripheral nerve tumours (malignant schwannoma) refractory to first-line therapy

Malignant peripheral nerve tumours (MPNT), e.g. malignant schwannoma, represent a relatively rare tumour entity which is usually regarded as a member of the group of soft-tissue sarcomas and treated accordingly. For chemotherapeutic approaches in metastasised disease, ifosfamide and doxorubicin have been identified as the most efficient agents, with overall response rates distinctly less than 50%. In case of non-response, recommendations for effective second-line regimens are lacking. We present the case histories of two patients with pulmonary metastasised MPNT that was primarily refractory to ifosfamide/doxorubicin. Both patients developed a partial remission with tumour reduction exceeding 50% after treatment with carboplatin in combination with etoposide (CE), 150 mg/m2 each, days 1-4 in 4-week intervals. Complete resectability of lung metastases could be achieved, with histologic evidence for advanced tumour regression at the time of resection. Patients remain in stable complete remission 20 and 28 months after surgery, respectively. Major CE-associated toxicity was cumulative myelosuppression, especially thrombocytopenia, reaching WHO grade 4. To our knowledge, this is the first report showing that CE is a possibly successful chemotherapeutic regimen in advanced MPNT, although further studies are necessary to evaluate its efficacy.

Postoperative Nomogram for 12-Year Sarcoma-Specific Death

PURPOSE: Few published studies have analyzed risk factors for sarcoma-specific death. We developed and internally validated a nomogram that combines the factors to predict the probability of 12-year sarcoma-specific death using a database of 2,136 prospectively followed adult patients treated at a single institution. PATIENTS AND METHODS: Nomogram predictor variables included age at diagnosis, tumor size (≤ 5, 5 to 10, or > 10 cm), histologic grade (high or low), histologic subtype (fibrosarcoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve tumor, synovial, or other), depth (superficial or deep), and site (upper extremity, lower extremity, visceral, thoracic or trunk, retrointraabdominal, or head or neck). Death from sarcoma or treatment complication was the predicted end point. Three prediction methods were compared, Kaplan-Meier analysis of all possible subsets, recursive partitioning, and Cox proportional hazards regression analysis. The concordance index was used as an accuracy measure with bootstrapping to correct for optimistic bias. RESULTS: Sarcoma-specific death at 12 years was 36% (95% confidence interval, 33% to 39%). The bootstrap-corrected concordance indices were as follows: Kaplan-Meier, 0.69; recursive partitioning, 0.74; and Cox regression, 0.77. A nomogram was drawn on the basis of the Cox regression model. This nomogram was internally validated using bootstrapping and shown to have excellent calibration. CONCLUSION: A nomogram has been developed to predict 12-year sarcoma-specific death. This tool may be useful for patient counseling, follow-up scheduling, and clinical trial eligibility determination.

Postoperative nomogram for 12-year sarcoma-specific death.

Few published studies have analyzed risk factors for sarcoma-specific death. We developed and internally validated a nomogram that combines the factors to predict the probability of 12-year sarcoma-specific death using a database of 2,136 prospectively followed adult patients treated at a single institution. Nomogram predictor variables included age at diagnosis, tumor size (< or = 5, 5 to 10, or > 10 cm), histologic grade (high or low), histologic subtype (fibrosarcoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve tumor, synovial, or other), depth (superficial or deep), and site (upper extremity, lower extremity, visceral, thoracic or trunk, retrointraabdominal, or head or neck). Death from sarcoma or treatment complication was the predicted end point. Three prediction methods were compared, Kaplan-Meier analysis of all possible subsets, recursive partitioning, and Cox proportional hazards regression analysis. The concordance index was used as an accuracy measure with bootstrapping to correct for optimistic bias. Sarcoma-specific death at 12 years was 36% (95% confidence interval, 33% to 39%). The bootstrap-corrected concordance indices were as follows: Kaplan-Meier, 0.69; recursive partitioning, 0.74; and Cox regression, 0.77. A nomogram was drawn on the basis of the Cox regression model. This nomogram was internally validated using bootstrapping and shown to have excellent calibration. A nomogram has been developed to predict 12-year sarcoma-specific death. This tool may be useful for patient counseling, follow-up scheduling, and clinical trial eligibility determination.

Pulmonary metastases from soft tissue sarcoma: analysis of patterns of diseases and postmetastasis survival.

To report the patterns of disease and postmetastasis survival for patients with pulmonary metastases from soft tissue sarcoma in a large group of patients treated at a single institution. Clinical factors that influence postmetastasis survival are analyzed. For patients with soft tissue sarcoma, the lungs are the most common site of metastatic disease. Although pulmonary metastases most commonly arise from primary tumors in the extremities, they may arise from almost any primary site or histology. To date, resection of disease has been the only effective therapy for metastatic sarcoma. From July 1982 to February 1997, 3149 adult patients with soft tissue sarcoma were admitted and treated at Memorial Sloan-Kettering Cancer Center. During this interval, 719 patients either developed or presented with lung metastases. Patients were treated with resection of metastatic disease whenever possible. Disease-specific survival was the endpoint of the study. Time to death was modeled using the method of Kaplan and Meier. The association of factors to time-to-event endpoints was analyzed using the log-rank test for univariate analysis and the Cox proportional hazards model for multivariate analysis. The overall median survival from diagnosis of pulmonary metastasis for all patients was 15 months. The 3-year actuarial survival rate was 25%. The ability to resect all metastatic disease completely was the most important prognostic factor for survival. Patients treated with complete resection had a median survival of 33 months and a 3-year actuarial survival rate of 46%. For patients treated with nonoperative therapy, the median survival was 11 months. A disease-free interval of more than 12 months before the development of metastases was also a favorable prognostic factor. Unfavorable factors included the histologic variants of liposarcoma and malignant peripheral nerve tumors and patient age older than 50 years at the time of treatment of metastasis. Resection of metastatic disease is the single most important factor that determines outcome in these patients. Long-term survival is possible in selected patients, particularly when recurrent pulmonary disease is resected. Surgical excision should remain the treatment of choice for metastases of soft tissue sarcoma to the lung.