Case Of Undifferentiated Pleomorphic Sarcoma Research Articles

A case of undifferentiated pleomorphic sarcoma of the thoracic wall poorly differentiated from local recurrence of lung cancer

A case of undifferentiated pleomorphic sarcoma of the thoracic wall poorly differentiated from local recurrence of lung cancer

A case of undifferentiated pleomorphic sarcoma in the neck

A case of undifferentiated pleomorphic sarcoma in the neck

Long-term effectiveness of uncemented allograft-prosthesis composite for reconstruction of bone defects after proximal femur tumor resection

To investigate the long-term effectiveness of uncemented allograft-prosthesis composite (APC) for reconstruction of bone defects after proximal femur tumor resection. Between June 2007 and March 2014, 21 patients who underwent uncemented APC reconstruction of proximal femur after tumor resection were retrospectively evaluated. There were 9 males and 12 females with an average age of 33.2 years (range, 19-54 years). There were 9 cases of giant cell tumor of bone, 5 cases of osteosarcoma, 4 cases of osteoblastic osteosarcoma, 2 cases of chondrosarcoma, and 1 case of undifferentiated pleomorphic sarcoma. Thirteen cases of benign bone tumors were all classified as stage 3 by Enneking staging; and 8 cases of malignant bone tumors were classified as grade ⅡB in 7 cases and grade ⅡA in 1 case according to the American Joint Committee on Cancer (AJCC) staging system. Among them, 7 patients underwent reoperation after recurrence, and the rest were primary operations; 8 patients presented with pathological fractures. The preoperative Harris hip score (HHS) and American Musculoskeletal Tumor Society (MSTS) score was 40 (30, 49) and 9.1±3.5, respectively. The length of osteotomy was 80-154 mm, with an average of 110 mm. At 1 year after operation and last follow-up, HHS and MSTS scores were utilized to evaluate the function of hip joint; the gluteus medius strength score was used to evaluation of the hip abduction function. Image examinations were taken at 1, 3, 6, 9, and 12 months after operation and every year thereafter to assess the union of allograft-host bone interfaces. Intra- and post-operative complications were also recorded. All patients were followed up 84-163 months (mean, 123.5 months). At 1 year after operation and last follow-up, the HHS and MSTS scores significantly improved when compared with the preoperative scores ( P<0.05). However, there was no significant difference in the HHS score, MSTS score, and gluteus medius strength score between the two time points after operation ( P>0.05). Image examination showed that all allograft-host bone interfaces achieved union after 5-10 months (mean, 7.6 months). At last follow-up, all patients had bone resorption, including 11 severe cases, 4 moderate cases, and 6 mild cases; the bone resorption sites included Gruen 1, 2, and 7 regions. Complications included 10 fractures and 1 prosthetic fracture. Local recurrence occurred in 3 patients and pulmonary metastasis in 3 patients. Uncemented APC is a reliable method for the reconstruction of bone defects after proximal femur tumor resection. It has the good long-term effectiveness and possesses obvious advantages in the union at the bone-bone surface.

A novel three dimensional-printed biomechanically evaluated patient-specific sacral implant in spinopelvic reconstruction after total en bloc sacrectomy.

Background: Reconstruction after a total sacrectomy is a challenge due to the special anatomical and biomechanical factors. Conventional techniques of spinal-pelvic reconstruction do not reconstruct satisfactorily. We describe a novel three-dimensional-printed patient-specific sacral implant in spinopelvic reconstruction after total en bloc sacrectomy. Methods: We performed a retrospective cohort study including 12 patients with primary malignant sacral tumors, including 5 men and 7 women with a mean age of 58.25years (range 20-66years), undergoing total en bloc sacrectomy with 3D printed implant reconstruction from 2016 to 2021. There were 7 cases of chordoma, 3 cases of osteosarcoma, 1 case of chondrosarcoma and 1 case of undifferentiated pleomorphic sarcoma. We use CAD technology to determine surgical resection boundaries, design cutting guides, and individualized prostheses, and perform surgical simulations before surgery. The implant design was biomechanically evaluated by finite element analysis. Operative data, oncological and functional outcomes, complications, and implant osseointegration status of 12 consecutive patients were reviewed. Results: The implants were implanted successfully in 12 cases without death or severe complications during the perioperative period. Resection margins were wide in 11 patients and marginal in one patient. The average blood loss was 3875mL (range, 2000-5,000mL). The average surgical time was 520min (range, 380-735min). The mean follow-up was 38.5months. Nine patients were alive with no evidence of disease, two patients died due to pulmonary metastases, and one patient survived with disease due to local recurrence. Overall survival was 83.33% at 24months. The Mean VAS was 1.5 (range, 0-2). The mean MSTS score was 21 (range, 17-24). Wound complications occurred in 2 cases. A deep infection occurred in one patient and the implant was removed. No implant mechanical failure was identified. Satisfactory osseointegration was found in all patients, with a mean fusion time of 5months (range 3-6months). Conclusion: The 3D-printed custom sacral prosthesis has been effective in reconstructing spinal-pelvic stability after total en bloc sacrectomy with satisfactory clinical outcomes, excellent osseointegration, and excellent durability.

A case of undifferentiated pleomorphic sarcoma in esophagus after multiple cancer treatments of surgery and chemoradiotherapy

BackgroundUndifferentiated pleomorphic sarcoma (UPS) in the esophagus is extremely rare. Therefore, there are few reports of UPS in the esophagus (UPSE). We present a case of UPSE after multiple cancer treatments.Case presentationA 73-year-old man with a history of cancer treatment, including distal gastrectomy, transverse colectomy, and chemoradiotherapy, was diagnosed with an elevated lesion such as a submucosal tumor in the lower esophagus by regular endoscopy. A boring biopsy was performed, and the specimen showed features of sarcoma. The patient underwent a partial esophagectomy without lymph node dissection. Histopathological findings confirmed an undifferentiated pleomorphic sarcoma. Adjuvant therapy was not administered, and the patient survived without recurrence 1 year after surgery.ConclusionsCurrently, complete resection is the only treatment option for UPSE. An optimal treatment strategy using chemotherapy or radiotherapy should be established.

A Case of Undifferentiated Pleomorphic Sarcoma of the Right Lower Extremity

Objective: To strengthen the understanding of the disease and treatment of undifferentiated pleomorphic sarcoma (UPS). Methods: The clinical data of an elderly male patient with UPS admitted to Hebei Provincial People’s Hospital in September 2020 were retrospectively analyzed, and relevant literatures were reviewed. Results: The patient was an elderly male, 74 years old. The color Doppler ultrasonography of the body surface mass showed a solid mass of subcutaneous soft tissue on the lateral side of the right thigh. Undirected arrangement, multinucleated tumor giant cells with strange shapes are seen, and mitotic figures are more common. Immunohistochemical staining: CKpan (-), Vimentin (+), Desmin (-), SMA (partial+), EMA (-), S100 (-), CD34 (vascular+), MyoD1 (-), Myogenin (-), Myoglobin (-), CD68 (-), Caldesmon (-), Ki-67 (about 40% active area). After six months of follow-up, no tumor recurrence was found. Conclusion: UPS is a rare malignant tumor in clinic. It has no specific clinical manifestations, imaging manifestations, histopathology and immunohistochemical staining. It is an exclusive diagnosis. Complete tumor resection is required, and pathological examination is the gold standard for diagnosis. Its long-term prognosis is relatively poor.

Efficacy and safety of a 3D-printed arthrodesis prosthesis for reconstruction after resection of the proximal humerus: preliminary outcomes with a minimum 2-year follow-up

BackgroundThe purpose of this study was to investigate the feasibility of using a three-dimensional (3D)-printed arthrodesis prosthesis for reconstruction of the proximal humeral defect after tumor resection.MethodsA novel proximal humeral prosthesis was designed to restore bone continuity and shoulder arthrodesis and was fabricated via 3D printing technology. Ten patients with primary malignancies in the proximal humerus underwent intra-articular resection and replacement with this prosthesis from 2017 to 2019. Baseline and operative data, oncological and prosthetic survival, and functional status were summarized.ResultsThis cohort consisted of 9 males and 1 female with a mean age of 32.1 ± 16.1 years. Diagnoses included 5 cases of osteosarcoma, 3 cases of chondrosarcoma and 1 each case of undifferentiated pleomorphic sarcoma and malignant myoepithelioma. The mean operative duration, intraoperative hemorrhage and postoperative length of hospitalization were 151.5 ± 61.0 min, 410.0 ± 353.4 ml and 5.3 ± 1.9 d, respectively. The mean follow-up duration was 29.3 ± 6.4 months, with a minimum of 24 months for the surviving patients. Two patients experienced local recurrence, and four patients developed distant metastases. Detachment of the taper occurred in two patients. One was managed conservatively, and the other received amputation due to concurrent tumor recurrence. The mean MSTS-93 and ASES scores and ranges of forwards flexion and abduction were 24.9 ± 3.1, 79.4 ± 8.3, 71.3 ± 19.4°, and 61.3 ± 16.4°, respectively. The functional outcomes were independent of the preservation of the axillary nerve. Histological study of the glenoid component showed evidence of bone ingrowth at the bone-prosthesis porous interface.ConclusionApplication of the 3D-printed arthrodesis prosthesis might be a safe and efficacious method for functional reconstruction in patients who underwent resection of the proximal humerus, especially for those without preservation of the axillary nerve.

Biomechanical analysis and effectiveness evaluation of zone Ⅰ+Ⅱ+Ⅲ reconstruction of hemipelvis with rod-screw prosthesis

To analyze the biomechanical properties of the rod-screw prosthesis based on a pelvic three-dimensional finite element model including muscle and ligament, and evaluate the effectiveness of zoneⅠ+Ⅱ+Ⅲ reconstruction of hemipelvis with rod-screw prosthesis in combination with clinical applications. A total of 21 patients who underwent hemipelvic tumor resection (zoneⅠ+Ⅱ+Ⅲ) and rod-screw prosthesis reconstruction between January 2015 and December 2020 were selected as the research subjects. Among them, there were 11 males and 10 females; the age ranged from 16 to 64 years, with an average age of 39.2 years. There were 9 cases of chondrosarcoma, 7 cases of osteosarcoma, 3 cases of Ewing sarcoma, and 2 cases of undifferentiated pleomorphic sarcoma. According to the Musculoskeletal Tumor Society Score (MSTS) staging, there were 19 cases of stage ⅡB and 2 cases of stage Ⅲ. Preoperative Harris Hip Score (HHS) and MSTS score were 54.4±3.1 and 14.1±2.0, respectively. Intraoperative 15 cases underwent extensive resection, 5 cases underwent marginal resection, and 1 case underwent intralesional resection. The CT image of 1 patient after reconstruction was used to establish a three-dimensional solid model of the pelvis via Mimics23Suite and 3-matic softwares. At the same time, a mirror operation was used to obtain a normal pelvis model, then the two solid models were imported into the finite element analysis software Workbench 2020R1 to establish three-dimensional finite element models, and the biomechanical properties of the standing position were analyzed. The operation time, intraoperative blood loss, and operation-related complications were recorded, and the postoperative evaluation was carried out with HHS and MSTS scores. Finally, the local recurrence and metastasis were reviewed. Finite element analysis showed that the peak stress of the reconstructed pelvis appeared at the fixed S1, 2 rod-screw connections; the peak stress without muscles was higher than that after muscle construction, but much smaller than the yield strength of titanium alloy. The operation time was 250-370 minutes, with an average of 297 minutes; the amount of intraoperative blood loss was 3 200-5 500 mL, with an average of 4 009 mL. All patients were followed up 8-72 months, with an average of 42 months. There were 7 cases of pulmonary metastasis, of which 2 cases were preoperative metastasis; 5 cases died, 16 cases survived, and the 5-year survival rate was 72.1%. There were 3 cases of local recurrence, all of whom did not achieve extensive resection during operation. The function of the affected limbs significantly improved, and the walking function was restored. The HHS and MSTS scores were 75.2±3.0 and 20.4±2.0 at last follow-up, respectively, and the differences were significant when compared with those before operation (t=22.205, P<0.001; t=11.915, P<0.001). During follow-up, 2 cases of delayed incision healing, 2 cases of deep infection, 1 case of screw loosening, and 1 case of prosthesis dislocation occurred, and no other complication such as prosthesis or screw fracture occurred. The stress and deformation distribution of the reconstructed pelvis are basically the same as normal pelvis. The rod-screw prosthesis is an effective reconstruction method for pelvic malignant tumors.

Undifferentiated pleomorphic sarcoma of the retroperitoneum mimicking a cortisol‐ and catecholamine‐secreting adrenal tumor

IntroductionRetroperitoneal tumors with endocrine abnormalities are suspected to be functional adrenal tumors. Retroperitoneal soft tissue sarcomas are rare tumors, without endocrine potential.Case presentationA 60‐year‐old male was referred for a 15 cm mass in the left suprarenal space. His plasma cortisol and catecholamine levels were elevated. He underwent open left adrenalectomy with radical nephrectomy and his endocrinological abnormalities were improved. Pathological findings suggested that it had originated from the retroperitoneal fat tissue, and a diagnosis of undifferentiated pleomorphic sarcoma was made based on the results of immunohistochemical analysis and fluorescence in situ hybridization. Interestingly, neither cortisol nor catecholamine was elevated when, 6 months after surgery, local recurrence developed.ConclusionThis is the first reported case of undifferentiated pleomorphic sarcoma accompanied by high levels of cortisol and catecholamine. We should keep in mind the possibility of tumors like retroperitoneal soft tissue sarcomas inducing endocrine abnormalities.

Diagnostic utility of ERG immunostaining in dermatofibroma

AimsDermatofibroma/fibrous histiocytoma (DF/FH) is a common cutaneous mesenchymal neoplasm exhibiting benign biological behaviour. However, the immunohistochemical utility of erythroblast transformation-specific-related gene (ERG) for diagnosing DF remains unknown. The authors reviewed...

A case of undifferentiated pleomorphic sarcoma of a retro-gastric origin, case report and review of literature.

Introduction and importanceUndifferentiated pleomorphic sarcoma (UPS) is one of the most common sarcomas affecting elderly patients, majority of UPS usually in the extremities, trunk, and retroperitoneum. However, its rarely observed in the digestive system. There are minimal data published regarding this topic.Case presentationHere we present a 58 years old female patient with a rare case of the pleomorphic sarcoma of retro-gastric mass measures about (24.7 × 23.5 × 17.4 cm) who underwent exploration laparotomy founded a freely and mobile retro-gastric mass measuring 30 × 20 cm with a cystic and solid component. The mass was freely and mobile located with no true gastric relation is the first one to be reported.Clinical discussionThe undifferentiated pleomorphic sarcomas or MFH has been regarded as the most common soft tissue sarcoma for many years (Kabashima et al., 2017). The incidence increases with age, with an inclusive incidence of about 1–2 cases per 100,000 patients per year, predominately 1.2:1 for males. Undifferentiated pleomorphic sarcoma of the stomach is rare and counted to have a worse prognosis when compared to the other types of pleomorphic malignant fibrous histiocytoma.ConclusionWe present a rare case of high-grade undifferentiated pleomorphic sarcoma of the retro-gastric mass with no invasion to the surrounding structures, managed with surgical resection of the tumor.

S1899 A Rare Case of a Primary Colonic Undifferentiated Pleomorphic Sarcoma in a Pediatric Patient

Introduction: Soft tissue sarcomas are a group of malignant tumors originating from primitive mesenchymal tissue. Rhabdomyosarcomas account for 4% of these tumors while the remaining types account for 3% of these tumors in children. Undifferentiated pleomorphic sarcoma is one of the many types of non-rhabdomyosarcoma malignancies and accounts for 2-6% of all childhood soft tissue sarcomas. They most commonly arise in the extremities. We present a pediatric patient with an undifferentiated pleomorphic sarcoma arising from the cecum. Case description/methods: A 12-year-old girl with a past medical history of obesity presented with intermittent abdominal pain, diarrhea, weight loss, and vomiting over a 4–5 month period. She was taking naproxen daily for the abdominal pain. She was also found to have iron deficiency anemia and anemia with a hemoglobin of 5.3 gm/dl. EGD was unremarkable but colonoscopy revealed a 7 cm fungating non-obstructing large mass in the cecum as well as 2 five mm pedunculated polyps in the rectum. Imaging revealed peri-colonic, mesenteric, and mediastinal lymph nodes as well as a 1.2 cm solid nodule in the left lower lobe. Then she underwent surgery revealing a colo-colonic intussusception and a right hemicolectomy for the malignancy. Later on, the patient had a video assisted thoracoscopic surgery with wedge resection of the pulmonary nodule. Colonic pathology revealed an undifferentiated pleomorphic sarcoma while lung pathology revealed histoplasmosis. Discussion: While rare pediatric malignancies such as this have a scarcity of literature presented, this appears to be the first reported case of an undifferentiated pleomorphic sarcoma arising from the cecum in a pediatric patient. Patients commonly present in the second decade of life with a painless mass but since this patient’s mass is in the intestines, it went undetected until it grew quite large ( > 7 cm). She did have typical symptoms of gastrointestinal malignancy such as vague abdominal pain, weight loss, bowel habit changes, and iron deficiency anemia but a malignancy is oftentimes lower on the differential for the pediatric age group. At the time of diagnosis, it is important perform staging via imaging including CT/MRI and PET. In our patient, when found, it had already metastasized to distant lymph nodes. Surgical resection of local disease can possibly lead to a relapse free life and possibly a cure but oftentimes adjuvant chemotherapy is also needed if there is metastasis.Figure 1.: A and B: Colonoscopy revealed a fungating non-obstructing, non-circumferential large mass in the cecum. The mass measured seven cm in length and 5 mm in diameter.

Rare Case of Undifferentiated Pleomorphic Sarcoma of the Right Parotid Gland Correlatively Demonstrated by 18F Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Contrast-Enhanced Magnetic Resonance Imaging.

Malignant fibrous histiocytoma (MFH)/undifferentiated pleomorphic sarcoma (UPS) is an uncommon malignancy in the head-and-neck region. UPS is a malignant neoplasm of uncertain origin that arises both in soft tissue and bone. We bring forth a 65-year-old female who presented with an ulceroproliferative growth in the right upper neck. Contrast-enhanced magnetic resonance imaging and 18F-fluorodeoxyglucose positron emission tomography–computed tomography revealed a large exophytic, solid-cystic growth in the right parotid region with locoregional extension and few ipsilateral lymph nodes. Biopsy of the lesion revealed UPS, which was previously called MFH. She was considered for chemoradiotherapy. Correlative imaging helps in adequate staging of large tumors with assessment of response to chemoradiotherapy.

Pazopanib in rare histologies of metastatic soft tissue sarcoma.

BackgroundUncommon histopathological subtypes account for less than 5% cases of soft tissue sarcoma (STS) and unclassified STSs comprise another 16%, these are often chemotherapy-resistant, with a dismal outcome in unresectable/metastatic disease. Prospective studies on the use of pazopanib in this cohort of patients are lacking in the literature. Here, we describe the safety and efficacy of pazopanib in rare histologies of advanced STS.Materials and methodsWe conducted a retrospective study at two tertiary cancer centres in India, evaluating 33 cases of rare subtypes of STS, who received pazopanib as per institutional protocol between January 2013 and December 2019. Patients who received pazopanib for unresectable/metastatic disease were enrolled in this study for clinicopathologic features, treatment outcome and evaluation of prognostic factors.ResultsOut of 33 patients, there were seven cases of undifferentiated pleomorphic sarcoma, four cases each of myxofibrosarcoma, epithelioid sarcoma and malignant peripheral nerve sheath tumour, three cases each of haemangiopericytoma and spindle cell sarcoma, two cases of haemangioendothelioma and a case each of clear cell sarcoma, retroperitoneal sarcoma, angiosarcoma and pleomorphic rhabdomyosarcoma-adult type. The objective response rate was 27%. Most of the patients (67%) received pazopanib in second or subsequent lines of therapy. The majority (70%) were started at a lower dose of 400/600 mg and only 43% of these (10/23) could be escalated to a full dose of 800 mg based on tolerance. On univariate analysis, pazopanib’s starting dose didn’t predict progression-free survival (PFS)/overall survival (OS)/response rate. At a median duration of follow-up of 18.8 months (range 1.9–150.4 months), the median PFS and median OS were 10.3 months (95% confidence interval (CI): 5.9–14.8) and 17.8 months (95% CI: 10.7–29.3), respectively. 27% of the patients experienced grade ¾ toxicities, 12% required dose modification of pazopanib and 21% needed permanent discontinuation due to toxicity.ConclusionOur study shows that pazopanib is active in rare subtypes of STS.

A Case of Undifferentiated Pleomorphic Sarcoma of the Maxillary Sinus

A Case of Undifferentiated Pleomorphic Sarcoma of the Maxillary Sinus

Breast Cancer and Secondary Cancer Recurrences After Autologous Tissue Reconstruction

BackgroundThe medical literature defining breast cancer recurrence and secondary cancers after autologous tissue reconstruction for breast cancer is sparse. We sought to identify and analyze occurrences at our institution. Patients and MethodsA 20-year retrospective review of cancer recurrences and atypical breast neoplasms after autologous tissue breast reconstruction at Roswell Park Comprehensive Cancer Center was conducted after being granted a waiver from the institutional review board. ResultsEighteen locoregional recurrences among 337 cases were identified and analyzed. Overall recurrence rate was 5.3%. Four secondary cancers (1.2%) were radiation-induced angiosarcoma, undifferentiated pleomorphic sarcoma, and metaplastic carcinoma. One case of flat epithelial atypia was identified. ConclusionOur retrospective review found incidence and survival after treatment of breast cancer concordant with reports in the literature. We also identified and analyzed secondary neoplasms, including a unique case of undifferentiated pleomorphic sarcoma and metachronous recurrence of breast carcinoma. A case of recurrence as metaplastic carcinoma was identified.

High grade pleomorphic sarcoma of penis: A rare clinical entity

The incidence of penile cancer has been reported to be around 0.005 % in males, with less than 5% of then being sarcomas among all types of penile malignancies Primary penile sarcoma is a rare disease that affects men of all ages. Different subtypes of primary penile sarcoma exist, with the rarest being pleomorphic sarcoma. Only few cases have been reported on pleomorphic sarcoma of penis with undifferentiated type being most commonly reported. No report on high grade pleomorphic sarcoma has been previously reported in the English literature. We report an extremely rare case of undifferentiated pleomorphic sarcoma of the penis in a 62-year-old patient who initially presented with a recurrent or residual penile lesion. The tumor was surgically excised, but the patient experienced local recurrence and died of metastatic disease 18 months after initial presentation. Keywords: Penile sarcoma penile cancer urogenital sarcoma.

Undifferentiated pleomorphic sarcoma of the right atrium in an infant

Undifferentiated pleomorphic sarcoma is a high-grade malignant tumour, accounting for one-third of all primary cardiac sarcomas. This paper describes a case of undifferentiated pleomorphic sarcoma in an infant using detailed morphological characterisation, immunohistochemical examination and a brief literature review. Taken together, these data will be useful not only for practicing pathologists but also for researchers of other biomedical fields for the development of procedures for differential diagnoses, investigation of the pathogenesis and development of therapeutic approaches for undifferentiated pleomorphic sarcoma.Received 4 February 2019. Revised 31 March 2020. Accepted 9 April 2020.Funding: The study did not have sponsorship.Conflict of interest: Authors declare no conflict of interest.Author contributionsDrafting the article: I.S. Murashov, E.E. KliverLiterature review: V.E. Kliver, T.A. AgeevaIllustrations: V.E. Kliver, A.M. VolkovCritical revision of the article: I.S. Murashov, E.E. KliverFinal approval of the version to be published: I.S. Murashov, V.E. Kliver, T.A. Ageeva, A.M. Volkov, E.E. Kliver

Undifferentiated Pleomorphic Sarcoma of the Thoracic Aorta Presenting with Ruptured Saccular Aneurysm: A Case Report

흉부 대동맥 미분화성 다형성 육종은 매우 드문 질환으로 색전 사례, 신혈관성 고혈압, 등 통증 등의 증상을 보일 수 있다. 특히 대동맥 벽에서 기원하여 소낭 동맥류 파열로 보고된 경우는 극히 드물며 진균성 동맥류나 관통성 죽상경화 궤양과 감별이 어렵다. 이에 저자들은 소낭성 동맥류 파열로 혈관 내 흉부 대동맥 치료를 시행한 후 종괴가 발생하여 수술적 생검으로 확진된 미분화성 다형성 육종의 전산화단층촬영과 양전자방출단층촬영 소견을 보고하고자 하며 이를 통해 정확한 진단과 적절한 치료에 도움을 주고자 한다.

Differing characteristics of cartilaginous lesions\xa0of the larynx

IntroductionThe tissues of the laryngeal region only rarely harbor primary cartilaginous lesions, and squamous cell carcinoma remains the most frequently encountered malignant tumor in this area.Materials and MethodsWe reviewed the salient histological features of cartilaginous laryngeal lesions to provide differential diagnostics and guidelines for distinguishing the benign from the malignant ones.ResultsCartilaginous neoplasms of the larynx include chondroma and chondrosarcoma. Among the overarching group of all forms of laryngeal sarcoma, chondrosarcoma forms the most common entity in the larynx, followed by rhabdomyosarcoma. Cartilaginous tumors comprise about 0.1%–1% of all laryngeal neoplasms with chondrosarcomas being more frequently encountered than chondromas. Several neoplasms earlier reported as giant-cell tumors of the larynx would most likely, using current terminology, be classified as cases of undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma, giant-cell variant) or aneurysmal bone cyst.ConclusionWhen true laryngeal sarcomas do exist, they may prove to be challenging lesions both for the pathologist and the treating clinician. The diagnostic problems are mainly a result of the infrequent exposure of clinicians and diagnosticians to these lesions.