Primary Pulmonary Myxoid Sarcoma and Thoracic Angiomatoid Fibrous Histiocytoma: Two Sides of the Same Coin?

Primary pulmonary myxoid sarcoma (PPMS) and thoracic angiomatoid fibrous histiocytoma (AFH) are rare neoplasms with EWSR1 fusions and overlapping morphology. Both tumor types often show epithelial membrane antigen expression, but AFH characteristically co-expresses desmin. We encountered a case of PPMS with the unexpected finding of patchy, strong anaplastic lymphoma kinase (ALK) (previously reported in AFH) and synaptophysin expression. We evaluated a cohort of PPMS and thoracic AFH with systematic morphologic comparison and surveyed for aberrant expression of ALK and synaptophysin. Medical records and slides were reviewed for 16 molecularly confirmed cases of PPMS (n=5) and thoracic AFH (n=11). Each case was scored for morphologic characteristics typical of PPMS and/or AFH. ALK, synaptophysin, chromogranin, desmin, and epithelial membrane antigen immunostains were performed on cases with available tissue. AFH and PPMS cases showed similar age at presentation and long-term tumor behavior. Almost all cases of PPMS and AFH had a fibrous pseudocapsule and lymphoid rim. All PPMS had myxoid stroma and reticular growth pattern, but these features were also present in a subset of AFH. Synaptophysin expression was present in 6 of 11 AFH and 1 of 5 PPMS; all tested cases were negative for chromogranin (n=15). One case of AFH and 1 case of PPMS showed focally strong coexpression of synaptophysin and ALK. AFH and PPMS show considerable clinicopathologic overlap. When supportive, the immunohistochemical findings described may aid in diagnosis before molecular confirmation. PPMS and AFH may be morphologic variants of the same clinicopathologic entity, which can show more immunophenotypic variability than previously reported.

Pulmonary artery angiomatoid fibrous histiocytoma mimicking pulmonary embolism.

A young man presented to the emergency department with pleuritic chest pain and shortness of breath. Of note, he recently went on a long-distance flight of about 9 hours. Given his recent long-distance travel and clinical symptoms, a pulmonary embolism was suspected. However, pathological examination of the excised pulmonary artery intraluminal mass demonstrated an angiomatoid fibrous histiocytoma. This case describes the clinicopathological and immunohistochemical features and molecular profile of a rare type of pulmonary artery tumour, a pulmonary artery angiomatoid fibrous histiocytoma.

Evidence of Chemoresponsiveness in Unresectable Metastatic Angiomatoid Fibrous Histiocytoma.

Angiomatoid fibrous histiocytoma (AFH) is a soft tissue neoplasm of intermediate biological potential. Typically a slow-growing tumor, it can recur locally. Rarely, it manifests as a soft tissue sarcoma capable of metastasis. When metastases are nonamenable to local therapy, it is believed uniformly fatal. We present 3 patients with metastatic AFH who demonstrated a sustained response to chemotherapy; including one who achieved complete remission with cryoablation. These cases reinforce the potential value of chemotherapy in some patients with unresectable metastatic AFH and provide the first case in the literature of cryoablation in AFH.

Angiomatoid fibrous histiocytoma in the spinal canal of T3-T4: a case report and literature review.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumour that occurs in the superficial tissue of extremities of children and young adults. A painless mass in the deep dermis and subcutaneous tissue is the main clinical manifestation. AFH also occurs infrequently in the central nervous system and is relatively common in the cranium. However, spinal canal AFH has not been described yet. We report a rare case of AFH in the cervical canal of a 20-year-old male patient. Microsurgical gross total resection of the tumour was performed, and the diagnosis was confirmed by postoperative pathology. To our knowledge, this is the first case of AFH in the spinal canal.

Surgical Management of Invasive Atypical Fibroxanthomas/Undifferentiated Pleomorphic Sarcomas of the Scalp: A Systematic Review Comparing Outer Table Resection to Full-Thickness Craniectomy.

Invasive atypical fibroxanthomas and undifferentiated pleomorphic sarcomas of the scalp are relatively rare spindle cell tumors that recur at high rates. Wide local excision and Mohs surgery alone are not feasible for the definitive management of lesions adhering to the underlying pericranium and/or calvarium. This brief clinical study presents 2 patient cases and includes a systematic review of the literature. In our literature review, 2 of 4 patients treated with outer table resection had no disease recurrence, 1 died due to an unrelated cause, and 1 died due to disease progression. Three of 9 patients treated with full-thickness craniectomy had no disease recurrence, 5 patient outcomes were not specified, and 1 died from disease progression. There are currently no recommendations for the management of these invasive scalp lesions. We believe invasive atypical fibroxanthomas/undifferentiated pleomorphic sarcomas of the scalp without frank calvarial involvement can be effectively treated with outer table resection.

Nodular fasciitis arising from the buccal region after segmentectomy with rapid growth mimicking postirradiation myxofibrosarcoma: A case report.

RATIONALE: Nodular fasciitis (NF) can be misdiagnosed as a sarcoma because of its rapid growth and pathological features, such as nuclear atypia and mitoses. PATIENT CONCERNS: We present a rare case of a 72-year-old Japanese man who developed NF with suspected postirradiation myxofibrosarcoma (MFS) after segmentectomy for left-sided osteoradionecrosis (ORN) of the mandible. DIAGNOSIS: A mass appeared in the intraoral postoperative wound 1 year after left-sided mandibular segmentectomy and showed rapid growth, reaching a size of 50 mm within 2 months. Incisional biopsy revealed strongly Ki-67-positive spindle-shaped cells with large irregular nuclei and a diagnosis of postirradiation MFS. INTERVENTIONS: The patient was diagnosed with oropharyngeal cancer (T4aN2bM0) and underwent surgical resection of primary oropharyngeal squamous cell carcinoma with selective neck dissection and reconstruction with a rectus abdominis musculocutaneous flap at the age of 57 years, followed by postoperative 66 Gy of radiotherapy combined with cisplatin administration. No recurrent or metastatic lesions of oropharyngeal squamous cell carcinoma have been detected for > 10 years. However, the ORN of the jaw worsened, and the patient underwent sequestrectomy 3 times on the right side of the mandible, followed by a left-sided segmentectomy at the age of 72 years. One year after segmentectomy, a 10-mm mass with soft-to-firm consistency appeared in the buccal mucosa of the wound and grew rapidly. An incisional biopsy revealed MFS. Complete resection under general anesthesia was immediately performed. OUTCOMES: The histopathological diagnosis of the excised specimen was NF without any malignant findings. Two years after surgery, there was no evidence of recurrence or metastasis. LESSONS: NF grows rapidly and has pathological features similar to sarcoma, making differential diagnosis difficult at the time of incisional biopsy. Further studies should be conducted to determine the clinical and pathological features of this tumor.

Undifferentiated high-grade pleomorphic sarcoma of the colon: a rare case report and literature review.

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma (MFH), hardly originates from the colorectum. CASE PRESENTATION: We reported a 65-year-old female presented with UPS in the descending colon. Computed tomography (CT) revealed an irregularly thickened descending colon. On colonoscopy examination, an ulcerative tumour was identified. The patient received radical resection of the left colon and partial enterectomy. The resected tumor was ulcerative, 10 cm × 8 cm × 5 cm in size, and infiltrated the serosa layer. Postsurgical pathology showed that the tumor was high-graded UPS in the colon with large amounts of necrotic tissues. CONCLUSIONS: UPS in the large intestine is a rare malignant tumor with a poor prognosis and unknown pathogenesis. The main treatment for UPS is early complete resection. Postsurgery adjuvant radiotherapy or chemotherapy can be attempted.

Angiomatoid Fibrous Histiocytoma Mimicking a Lymph Nodal Lesion: A Case Report.

Angiomatoid fibrous histiocytoma is an uncommon soft tissue neoplasm with potential for recurrence and rare metastasis. The majority of cases are painless, slow growing and occur in superficial extremities of young adults. Here we report a case of Angiomatoid fibrous histiocytoma in a 28-year-old male patient presenting as a slowly growing painful mass in the groin region. This case is of particular interest due to its uncommon site of presentation and its misdiagnosis as lymph nodal lesion on radiology. Although it is a rare entity, it should be considered in differential diagnosis of soft tissue mass in a young patient.

Paraneoplastic syndrome due to angiomatoid fibrous histiocytoma: a known presentation of an uncommon diagnosis in a rare site and age.

A woman in her 70s presented with a small subcutaneous retrosacrococcygeal mass and a history of elevated erythrocyte sedimentation rate present for several years. It was misdiagnosed as an inflammatory process of unclear origin. She underwent further investigation with the appearance of weight loss and weakness. A sacrococcygeal mass was noted on CT scan. A core needle biopsy was inconclusive for chordoma versus myoepithelioma. Wide surgical resection of the tumour including the coccygeal bone was performed. Following surgery, all the systemic symptoms resolved with normalisation of inflammatory markers. The pathological examination showed a relatively circumscribed multinodular myxoid tumour with lymphatic tissue cuff. Pan-sarcoma fusion analysis detected an EWSR1 (Exon7)-CREB1 (Exon7) fusion gene. The lesion was diagnosed as angiomatoid fibrous histiocytoma with paraneoplastic syndrome presentation of several years' duration.

Malignant fibrous histiocytoma of the floor of mouth: a case report and review of the literature.

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma in late adulthood and usually occurs in the limbs, trunk, and peritoneum. Less than 10% of MFH cases occur in the head and neck region. The clinical manifestations and pathological features of MFH are atypical, and it is difficult to make a clinical diagnosis. We describe a rare case of MFH of the floor of mouth and provide our diagnosis and treatment experiences. Through this review, we also evaluate the origin, World Health Organization (WHO) classification, clinical presentations, pathological features, treatment methods, and prognosis of MFH. MFH may originate from fibroblasts or primitive mesenchymal cells. MFH was defined as undifferentiated pleomorphic sarcoma in the 2002 WHO classification of bone and soft tissue tumors. The most common manifestation of MFH is a painless enlarging nodule, often without overlying epidermal ulcers. Jaw lesions are usually found after displays of swelling, pain, paresthesia, and loose teeth. MFH is composed of pleomorphic spindle cells, usually with hemorrhage, necrosis, and lymphocyte infiltration. The main treatment method is surgical resection. Moreover, radiotherapy and chemotherapy have certain auxiliary effects. The local recurrence and distant metastasis of MFH are common, and the prognosis is poor. Therefore, determining the histopathological features of MFH and conducting appropriate immunohistochemical examinations are crucial in establishing the correct diagnosis. In-depth study is required in order to have a better understanding of head and neck MFH.

Fibrohistiocitoma maligno de face: uma apresentação rara / Malignant fibrohistiocytoma of face: a rare presentation

Contexto: O fibrohistiocitoma maligno é um sarcoma de tecidos moles muito agressivo, com rara apresentação limitada à pele e tecido subcutâneo em face. O diagnóstico é anatomopatológico com auxílio da imuno-histoquímica. Descrição do caso: Este artigo relata o caso de um paciente com diagnóstico de fibrohistiocitoma maligno restrito à face com boa resposta terapêutica após exérese cirúrgica. Discussão: Tendo em vista a raridade dessa afecção, dificuldade diagnóstica devido ao quadro inespecífico e com rápida evolução, é importante lembrar desse possível diagnóstico e atuar precocemente. Conclusões: O diagnóstico precoce interfere de forma significativa na evolução do quadro, sendo necessária a manutenção do acompanhamento oncológico e dermatológico com o intuito de detectar precocemente recidivas locais e metástases a distância.

Primary Pulmonary Undifferentiated Pleomorphic Sarcoma: A Rare Malignant Lung Tumor.

We report a case of a 56-year-old man with persistent right upper lobe cavitary mass. A chest computed tomography scan showed about 4-cm-sized mass with internal low attenuation and peripheral enhancement in right upper lobe apical segment. The lesion size increased over 1 month. Right upper lobectomy was performed with the intention to treat the lesion. Pathological examination showed primary pulmonary undifferentiated pleomorphic sarcoma. We describe this rare lung disease to remind that primary pulmonary undifferentiated pleomorphic sarcoma could be the differential diagnosis of pulmonary cavitary mass lesions.

How Can We Differentiate Local Recurrence From Heterotopic Ossification After Resection and Implantation of an Oncologic Knee Prosthesis in Patients with a Bone Sarcoma?

BACKGROUND: Heterotopic ossification (HO) is common after total joint arthroplasty and usually does not cause diagnostic problems. However, the occurrence of HO after oncologic prostheses implantation can be troublesome as it may mimic a locally recurrent tumor. Because this distinction could have a profound impact on the surgeon and patient, it is important to distinguish the two entities; to our knowledge, no study has evaluated this after oncologic endoprosthetic reconstruction around the knee after tumor resection. QUESTIONS/PURPOSES: (1) How common is the occurrence of HO compared with local recurrence (LR) after resection of bone sarcoma and the use of an oncologic knee prosthesis? (2) Are there any factors associated with the development of HO after limb salvage procedures with an endoprosthesis? (3) What features allow the surgeon to differentiate HO from a locally recurrent tumor in this setting? METHODS: Between 2002 and 2018, we performed 409 resections of primary bone tumors followed by reconstructions with oncologic endoprostheses. Of these, 17% (71 of 409) died before 2 years and did not have HO at that time, 2% (8 of 409) were lost to follow-up before 2 years, and another 2% (10 of 409) did not have radiographs available at a minimum of 2 years after surgery (and had not developed HO before then), and so could not be analyzed, leaving 320 patients for analysis in this retrospective study. Forty-two patients were excluded; 2% (5 of 320) for a history of failed allograft reconstruction, 3% (8 of 320) for pathologic fracture at presentation, 2% (6 of 320) for inadequate or complicated biopsy, 1% (2 of 320) for stem fractures, 2% (7 of 320) for stem loosening, and 4% (14 of 320) for extracortical bone bridging, leaving 278 patients for final evaluation. Two observers analyzed AP and lateral radiographs for signs of HO at a mean follow-up of 63 ± 33 months after surgery. We defined HO as extraskeletal bone formation in soft tissues. The primary study endpoint was survivorship free from HO, as ascertained by a competing-risks estimator. To identify factors associated with HO appearance, the demographic, radiographic, clinical, pathologic, and surgical characteristics were compared between patients with HO and those who had no lesion. Characteristic features were also compared between patients with HO and those with LR to help their differentiation. Univariate analysis was used for all statistical evaluations. RESULTS: HO developed in 8% (21 of 278) of patients in whom oncologic knee prosthesis was implanted. LR developed in 10% (28 of 278) of the patients. According to survivorship estimates, the HO-free survival rate was not different from the LR-free survival rate at 2 years after oncologic knee reconstruction (76 ± 5% [95% CI 63 to 87] versus 74 ± 5% [95% CI 62 to 88]; p = 0.19). History of infection was more common in patients with HO than in patients with no lesion (19% [4 of 21] versus 5% [12 of 229], Odds ratio [OR] 6 [95% CI 2 to 17]; p < 0.001). The male sex was more common in the HO group as well (76% [16 of 21] versus 55% [128 of 229], OR 2 [95% CI 1 to 5]; p = 0.03). The Modular Universal Tumor and Revision System prosthesis was more frequently used in patients with HO (67% [14 of 21]) compared to those with no lesions (40% [92 of 229]; OR 2 [95% CI 1 to 5]; p = 0.02). The lesion border in radiographs was ill-defined in 19% (4 of 21) of patients with HO and 100% (28 of 28) of patients with LR (OR 8 [95% CI 3 to 20]; p < 0.001). The median time to the appearance of HO was shorter than the time to LR (8 months [3 to 13] versus 16 months [11 to 21], [95% CI 10 to 13]; p < 0.001). Pain at presentation was more frequent in patients with LR than in those with HO (86% [24 of 28] versus 14% [3 of 21], OR 36 [95% CI 7 to 181]; p < 0.001). CONCLUSION: HO may occur after the use of oncologic knee prostheses for reconstruction after tumor resection. In most patients, HO could be differentiated from local recurrence through identifying a well-defined border on radiographs. Otherwise, factors such as an earlier time of presentation and absence of pain could suggest an HO, rather than an LR. LEVEL OF EVIDENCE: Level III, therapeutic study.

Undifferentiated pleomorphic sarcoma of skin in unusual locations: Report of two cases.

Undifferentiated pleomorphic sarcoma (UPS) of the skin is a rare soft tissue sarcoma subtype with a high risk of metastasis and local recurrence. Ultraviolet exposure plays a prominent role in its etiology. Herein, we present two rare cases of UPS of the skin with an occurrence in non-ultraviolet exposed locations and discuss the need of wide resection and the identification of the depth of the tumor. Due to the resection of the skin, resulting defect was undertaken with skin grafting. One case was extending to the fascia and, after the resection, vacuum-assisted closure therapy was used for the formation of granulation tissue. No recurrence was seen after three and four years for either cases. In conclusion, UPS is a rare, aggressive malignant tumor with a high local recurrence and metastasis rate. Suspicion of malignancy in a plaque-like or nodular lesion in the skin is crucial, and local recurrence and metastasis risk is significantly correlated to the success of wide excision.

Finn/Orthopaedic Salvage System Distal Femoral Rotating-Hinge Megaprostheses in Oncologic Patients: Long-Term Complications, Reoperations, and Amputations.

BACKGROUND: There is a lack of evidence regarding long-term outcomes of rotating-hinge knee prostheses with distal femoral replacement in a large oncologic patient series. In this study, we investigated the proportion of patients experiencing complications requiring surgery in the long term, as well as the cumulative incidence of implant removal/revision and amputation at 5, 10, 15, and 20 years through competing risk analyses. METHODS: We retrospectively studied 214 patients treated with a Finn/Orthopaedic Salvage System (OSS) knee prosthesis (Zimmer Biomet) after distal femoral resection from 1991 to 2017. The study end points were postoperative complications requiring surgery. Reoperations were classified as major when there was (1) removal of the metal-body femoral component, the tibial component, or the bone-implant fixation; (2) major revision (exchange of the metal-body femoral component, the tibial component, or the bone-implant fixation); or (3) amputation. Minor reoperations were defined as all other reoperations. Competing risk analysis was used to estimate the cumulative incidence of implant removal/revision or amputation. RESULTS: There were 312 reoperations in 113 patients (98 major reoperations in 68 patients and 214 minor reoperations). Seventeen patients (8%) required ≥5 additional operations, and 21 patients (10%) required >1 major reoperation. Although the number of reoperations decreased over time, major and minor reoperations continuously accrued after 10 years. The cumulative incidences of implant removal or revision for any reason at 5, 10, 15, and 20 years were 22.6%, 30.1%, 34.3%, and 42.5%, respectively. Although most implant removals/revisions occurred in the first 10 years, the risk persisted after 10 years, at a mean of 1.24%/year, mainly due to deep infection (1.06%/year). CONCLUSIONS: The long-term outcomes of treatment with a Finn/OSS distal femoral rotating-hinge knee prosthesis showed it to be a durable reconstruction technique. The rate of implant removal/revisions after 10 years was gradual (1.24%/year). Deep infection remains a major late-failure mechanism, and lifetime surveillance for prosthetic problems is needed. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.