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Surgical Pathology Clinics Dec 2021Diagnosis of osteosarcoma can be challenging because of its diverse histological patterns and the lack of diagnostic biomarkers for most examples. This review summarizes... (Review)
Review
Diagnosis of osteosarcoma can be challenging because of its diverse histological patterns and the lack of diagnostic biomarkers for most examples. This review summarizes the key pathologic findings of osteosarcoma subtypes (high-grade central, parosteal, low-grade central, periosteal, high-grade surface, and secondary) with an emphasis on describing and illustrating histological heterogeneity to help general pathologists. Differential diagnoses are listed for each entity, and histological subtype and distinguishing features, including molecular genetic findings (eg, MDM2, IDH, H3F3A, FOS, and USP6), are discussed. The review also covers recently established and emerging concepts and controversies regarding osteosarcoma.
Topics: Bone Neoplasms; Diagnosis, Differential; Humans; Osteosarcoma
PubMed: 34742481
DOI: 10.1016/j.path.2021.06.003 -
Case Reports in Oncology 2021Osteosarcoma is the most common malignant primary bone tumor that occurs most frequently in the second decade of life but rarely in patients over 40 years of age. The...
Osteosarcoma is the most common malignant primary bone tumor that occurs most frequently in the second decade of life but rarely in patients over 40 years of age. The most common primary sites of osteosarcoma are the distal femur followed by proximal tibia and proximal humerus, and involvement of the wrist is extremely rare. Moreover, dedifferentiated osteosarcoma is also a rare condition that progresses to high-grade osteosarcoma from low-grade osteosarcoma, usually central low-grade osteosarcoma or parosteal osteosarcoma that bears MDM2 and/or CDK4 gene amplifications. We herein report an extremely rare case of dedifferentiated osteosarcoma arising in the distal ulna of an adult over 40 years of age. The patient was a 46-year-old man with a 2-month history of pain in his left swollen wrist. The initial radiological findings suggested a benign bone tumor in the distal ulna, and the lesion was marginally excised at the nearby hospital. Although the pathological diagnosis at the nearby hospital suggested a benign cartilaginous tumor, the tumor recurred in an aggressive manner 8 months after the initial surgery. The patient was referred to our hospital, and an incisional biopsy showed a high-grade osteosarcoma. The primary tumor was retrospectively re-evaluated at our hospital and diagnosed as low-grade osteosarcoma. Since neoadjuvant chemotherapy failed to shrink the tumor, the patient had to undergo below the elbow amputation to cure the disease. Although the tumor was negative for MDM2 nor CDK4, the definitive diagnosis of dedifferentiated osteosarcoma was made according to the clinical course and the histological findings. Lung metastases were found 10 months after the amputation, which were successfully treated by neoadjuvant chemotherapy and surgery. The patient has been doing well with no evidence of disease for 1 year and 6 months. Surprisingly, the literature review revealed that many low-grade osteosarcomas of the distal ulna progressed to high-grade dedifferentiated osteosarcomas. One should bear in mind that the diagnosis and treatment for bone-forming tumors of the distal ulna should be made very carefully because, although rare, it is possible that the tumor may initially appear as a benign or low-grade malignant tumor and may progress to high-grade osteosarcoma.
PubMed: 34703440
DOI: 10.1159/000518266 -
Cureus Sep 2021Osteomas are most common among all primary bone tumors of skull bones. They are usually asymptomatic due to their small size and slow growth. They are found incidentally...
Osteomas are most common among all primary bone tumors of skull bones. They are usually asymptomatic due to their small size and slow growth. They are found incidentally on imaging studies for other neurologic symptoms. Osteoma may be single or multiple when present. They should be differentiated from meningiomas, chordomas, schwannomas, and parosteal osteosarcoma by using different diagnostic methods, including histopathologic study. During routine dissection for MBBS students in an 87 years old female cadaver, we found multiple (seven in number) irregular, lobulated bony masses/structures. Their positions were different with respect to the layers of meninges. Some were present between the dura mater and arachnoid mater compressing the adjacent brain tissues forming impressions on them, and some were outside the dura mater. So, into the previously existing classification, we want to add a new variety under the type b category, i.e., mixed type (intraparenchymal, dural, skull vault) as pointed under the subtype V, which is found in our case.
PubMed: 34659951
DOI: 10.7759/cureus.17737 -
Frontiers in Veterinary Science 2021This case report describes a rare form of malignant bone tumor in an 8-year-old Labrador retriever. This dog initially presented for evaluation of a right distal humeral...
This case report describes a rare form of malignant bone tumor in an 8-year-old Labrador retriever. This dog initially presented for evaluation of a right distal humeral mass. Radiographs of the right elbow and thorax were performed, revealing a smooth mineralized mass adjacent to the lateral aspect of the distal humerus and a 5mm pulmonary nodule. Computed tomography (CT) of the humerus and thorax showed a smooth mineralized lesion adjacent to the lateral humeral epicondyle, and a right cranial lung lobe nodule with a thin mineral rim. Surgical biopsies of both lesions were diagnostic for parosteal osteosarcoma (POSA). The dog was then treated with stereotactic body radiation therapy (SBRT) which controlled the dog's discomfort for 14 months until he became progressively painful and subsequently had his right forelimb amputated. This case report is the first to document the CT imaging characteristics of a metastatic appendicular POSA in a dog and the first dog described with POSA treated with SBRT. The dog lived for 623 days after histopathologic diagnosis and 849 days after initial presentation with pulmonary metastatic disease.
PubMed: 34504888
DOI: 10.3389/fvets.2021.715908 -
Case Reports in Oncology 2021Osteosarcoma is the most common primary bone cancer in all age groups. Metastasis mostly occurs with high-grade tumors disseminating to the lungs and other bones. Spread...
Osteosarcoma is the most common primary bone cancer in all age groups. Metastasis mostly occurs with high-grade tumors disseminating to the lungs and other bones. Spread to the pancreas is rare and undocumented in the low-grade subtypes. Additionally, it is uncommon for the disease course of low-grade subtypes to involve multiple relapses. We present a 35-year-old woman with parosteal osteosarcoma who has experienced an atypical metastasis to the pancreas as well as multiple local and pulmonary relapses. The lesion was identified incidentally on routine imaging, and the patient underwent resection. We compare our case to the other reports of pancreatic metastasis in the literature. Despite being especially rare, clinicians ought to be aware of pancreatic metastasis of osteosarcoma. Furthermore, despite parosteal osteosarcoma's less aggressive disease course, it can uncommonly lead to multiple relapses. We present a rare case exemplifying these phenomena in the prognostically favorable histologic subtype of parosteal osteosarcoma.
PubMed: 34188664
DOI: 10.1159/000516033.2021.1.test -
Journal of Surgical Oncology Sep 2021The subtypes of surface osteosarcomas include well-differentiated, low-grade parosteal osteosarcoma (POS), intermediate-grade periosteal osteosarcoma (PerOS), high-grade...
BACKGROUND AND OBJECTIVES
The subtypes of surface osteosarcomas include well-differentiated, low-grade parosteal osteosarcoma (POS), intermediate-grade periosteal osteosarcoma (PerOS), high-grade surface osteosarcoma (HGSO), and high-grade, dedifferentiated POS (dPOS). We aimed to determine disease progression, defined as local recurrence and metastatic disease, and overall (OS) and disease-specific survival (DSS). We identify outcome predictive factors and report functional results.
METHODS
This retrospective study evaluated patients with primary surface osteosarcoma at our hospital from 1992 to 2019. Fifty-one patients had a median follow-up of 6.1 years (range: 0.1-25.2). Histologic subtypes included 32 POS, 11 PerOS, 4 HGSO, and 3 dPOS. Bone and soft tissue margins were classified using the American Joint Committee on Cancer residual tumor classification (Rx = Not evaluable; R0 = negative margin; R1 = microscopic positive margin; and R2 = macroscopic positive margin) and the modified R classification (mRx = not evaluable; mR0 = negative margin >1 mm; mR1 = negative margin ≤1 mm; mR1-dir: Positive microscopic margin locally; mR2a: Positive macroscopic margin locally; mR2b: positive macroscopic margin distally; and mR2C: positive macroscopic margin locally and distally). Forty-one patients had functional outcomes.
RESULTS
Three POS patients developed recurrence: two had R0 margins and one an intralesional resection. Five patients developed lung metastases (POS: 3, dPOS: 2). Four patients died. The only significant disease progression predictor was age. OS at 10 years was 97%. 48 patients had negative bone margins (R0 or mR0 and mR1) and 47 patients had negative soft-tissue margins (R0 or mR0 and mR1). The average MSTS score was 88.43 (range: 34.29-100).
CONCLUSIONS
We advocate surgery for POS and believe R0 (mR0 and mR1 resections) or planned R1 (mR1-dir) to preserve function are acceptable. We favor chemotherapy and surgery for PerOS, though a chemotherapeutic response is highly variable. High-grade tumors are the most infrequent subtype, but HGSO and dPOS seem to portend a poorer prognosis. Good function can be obtained.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Bone Neoplasms; Child; Female; Follow-Up Studies; Humans; Limb Salvage; Lymphatic Metastasis; Male; Margins of Excision; Middle Aged; Neoplasm Recurrence, Local; Osteosarcoma; Prognosis; Retrospective Studies; Risk Factors; Survival Rate; Time Factors; Young Adult
PubMed: 34043244
DOI: 10.1002/jso.26531 -
Veterinary and Comparative Oncology Mar 2022While the majority of canine osteosarcomas (OSA) arise from the medullary cavity, a subset arises from the surface of bone. In humans, surface OSA often has a more...
While the majority of canine osteosarcomas (OSA) arise from the medullary cavity, a subset arises from the surface of bone. In humans, surface OSA often has a more indolent disease course with better outcomes than medullary OSA. The aim of this retrospective case series was to evaluate the clinical outcome and potential prognostic factors of dogs with surface OSA. Medical records from 11 dogs previously diagnosed with surface OSA were included. Histopathology of cases was evaluated during case review by two veterinary anatomic pathologists. Median progression free interval (PFI) and overall median survival time (OST) were estimated using Kaplan-Meier methods. Intergroup comparisons were performed using log-rank tests. Six dogs were diagnosed with periosteal OSA, 4 dogs with parosteal OSA, and one dog with an unclassified surface OSA. Two dogs were found to have metastatic disease at the time of diagnosis and four developed metastatic lesions after treatment. The median PFI and median OST for all dogs with surface OSA was 425 and 555 days, respectively. The 6 dogs diagnosed with periosteal OSA had a median PFI of 461 days and median OST of 555 days, while the 4 dogs with parosteal OSA had a PFI of 350 days and the OST could not be calculated. Multiple prognostic factors (surgery, systemic adjunctive therapy, elevated alkaline phosphatase at diagnosis, appendicular vs axial location, mitotic count, and tumour grade) were evaluated and none were prognostic for PFI or OST. Dogs with surface OSA appear to have prolonged PFI and OST, consistent with humans with surface OSA.
Topics: Animals; Dogs; Bone Neoplasms; Dog Diseases; Osteosarcoma; Retrospective Studies
PubMed: 34033204
DOI: 10.1111/vco.12741 -
Clinical and Experimental Dental... Dec 2021Primary osteosarcoma of the jaw bones is very rare, and histological features of gnathic osteosarcoma remain obscure. The purpose of this study was to describe the...
OBJECTIVE
Primary osteosarcoma of the jaw bones is very rare, and histological features of gnathic osteosarcoma remain obscure. The purpose of this study was to describe the clinicopathological features of gnathic osteosarcoma.
MATERIALS AND METHODS
Seven cases of gnathic osteosarcoma from Japan diagnosed during the period between 2000 and 2016 were examined retrospectively. The histology of the surgical pathology materials was reviewed by two pathologists. Clinical information was obtained from the hospital's information system.
RESULTS
Of the seven cases, two patients had secondary osteosarcomas. As for the five cases of primary osteosarcoma, their ages ranged from 26 to 58 years (mean: 36.2, median: 28). Histologically, three cases were fibrotic tumors composed of spindle-shaped cells with mild to moderate nuclear atypia and the collagenous stroma accompanied by woven bones or mature lamellar-like bones. Two cases had cartilage formation. MDM2 and CDK4 expression was observed in two out of three cases on immunostaining. The histopathology of these three cases was regarded as the counterpart of low-grade osteosarcomas, namely, parosteal osteosarcoma and low-grade central osteosarcoma, arising in long bones.
CONCLUSIONS
The surprisingly high incidence (60%, 3/5 cases) of low-grade osteosarcoma explains the reason why gnathic osteosarcomas present a more favorable prognosis than osteosarcomas arising in long bones. Furthermore, it provides insight into the tumorigenesis mechanism of low-grade osteosarcomas arising in the jaw and other sites.
Topics: Adult; Bone Neoplasms; Humans; Middle Aged; Osteosarcoma; Prognosis; Proto-Oncogene Proteins c-mdm2; Retrospective Studies
PubMed: 34008925
DOI: 10.1002/cre2.442 -
International Journal of Oral and... Dec 2021'Bullough lesions', also referred to as protuberant fibro-osseous lesions (PFOL), are rare temporal bone lesions initially described in 1999. Since only 12 cases have... (Review)
Review
'Bullough lesions', also referred to as protuberant fibro-osseous lesions (PFOL), are rare temporal bone lesions initially described in 1999. Since only 12 cases have been reported, several key issues, such as their origin and recommended management strategies, remain unresolved. This article reports the largest cohort included in the literature to date, comprising four patients with PFOL. PFOL appears to be characterized by female and right-side predominance. These lesions were consistently located regarding the mastoid, generally diagnosed in early adulthood, without functional symptoms, and were always fibro-osseous. Invasive/malignant features were not found on imaging or histology. The main differential diagnosis was malignant low-grade parosteal osteosarcoma. Clinical examination and computed tomography images provided strong elements supporting the diagnosis of PFOL. Biopsy allowed molecular biology investigations (MDM2 and CDK4 amplification), in order to rule out low-grade parosteal osteosarcoma.
Topics: Adult; Biopsy; Bone Neoplasms; Bone and Bones; Diagnosis, Differential; Female; Humans; Temporal Bone; Tomography, X-Ray Computed
PubMed: 33865660
DOI: 10.1016/j.ijom.2021.03.002 -
The American Journal of Case Reports Apr 2021BACKGROUND Osteoma is a benign bone tumor that typically arises in facial bones and less frequently in the long bones. It rarely occurs in the appendiceal skeleton....
BACKGROUND Osteoma is a benign bone tumor that typically arises in facial bones and less frequently in the long bones. It rarely occurs in the appendiceal skeleton. Pathologic findings are similar to those for parosteal osteosarcoma, periostitis ossificans, and osteoid osteoma. Diagnosing osteoma at an unusual site is always problematic. Here, we present a case of costal osteoma that was found incidentally on screening and produced mild symptoms in the patient. CASE REPORT A 53-year-old man was examined because of a rib mass in the eighth rib on his left side, which had been present for 2 years. A computed tomography scan revealed that the intensely dense mass arose from the external surface of the eighth rib. Microscopic examination showed that the lesion consisted of mature lamellar bone with several Haversian systems, typical of an osteoma. No atypical spindle cells or necrosis were identified. The diagnosis was osteoma. CONCLUSIONS Because the anatomy of the ribs poses a challenge when performing needle biopsy, diagnosing bony lesions solely based on that technique is difficult. The diagnosis of costal osteoma should be made carefully, based on clinical, radiological, and pathological findings. To the best of our knowledge, ours is the first case report about a costal osteoma. It is useful for chest surgeons, pathologists, and radiologists as an example of a rare presentation of this tumor.
Topics: Biopsy; Bone Neoplasms; Humans; Male; Middle Aged; Osteoma; Osteoma, Osteoid; Ribs; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 33864678
DOI: 10.12659/AJCR.930902