Uncommon Thigh Mass in Neurofibromatosis Type 1: Unveiling Aggressive Epithelioid Sarcoma.

Background: Patients with neurofibromatosis type I (NF1) have an increased risk of developing soft-tissue sarcomas, particularly those related to the nervous system. Epithelioid sarcoma (ES) is an exceptionally rare subtype of soft-tissue sarcoma, with limited knowledge about its clinical presentation and optimal management in NF1. This report aims to provide insights into the characteristics and outcomes of ES in NF1 patients. Case description: A 37-year-old man with a history of NF1 presented with a progressively worsening mass on his right inner thigh. An MRI scan revealed a well-defined tissue mass originating from the adductor magnus muscle, later confirmed as ES through histopathology and immunohistochemistry. Considering poor local and general prognosis, the multidisciplinary team recommended salvage hip disarticulation, however the patient refused and opted for palliative marginal resection to reduce the tumour size. The patient's condition declined rapidly, and he succumbed six days after the surgery. Conclusion: This case highlights the rarity of ES in NF1 patients and underscores the potential for malignant tumour development in this population. Further research is needed to improve our understanding and management of sarcomas in the context of NF1. LEARNING POINTS: Patients with neurofibromatosis type 1 or von Recklinghausen's disease have a higher risk than those with other types of neurofibromatosis of developing benign or malignant soft-tissue tumours especially related to the nervous system.Epithelioid sarcoma is an extremely rare subtype of soft-tissue sarcoma and is exceptionally associated with neurofibromatosis type 1.A multidisciplinary approach remains essential in the diagnosis, management, and treatment of soft-tissue sarcomas in patients with neurofibromatosis type 1.

Primary extrahepatic hydatid cyst of the thigh, an unusual presentation of sciatica: A case report of a diagnostic challenge.

INTRODUCTION: Sciatica, a condition characterized by pain along the sciatic nerve distribution, is commonly associated with nerve compression or irritation. However, its etiology can vary, including rare non-spinal causes such as hydatid cysts. We present a case of hydatid cyst in the thigh causing sciatica-like symptoms, highlighting the diagnostic challenges and management approach. CASE PRESENTATION: A 40-year-old patient with a history of pulmonary tuberculosis presented with persistent lumbosciatic pain despite conservative treatment. Physical examination revealed left sciatica without spinal abnormalities. MRI revealed a hydatid cyst in the thigh, causing nerve irritation. Surgical resection of the cyst was performed, achieving symptom resolution. DISCUSSION: Hydatid cysts in skeletal muscles are rare, with atypical presentations complicating diagnosis. Localization in the thigh, particularly the biceps femoris muscle, is uncommon. Diagnostic modalities include imaging and serological tests, while treatment involves surgical excision and postoperative albendazole therapy. CONCLUSION: Recognition of rare presentations like thigh hydatid cysts causing sciatica-like symptoms is crucial for timely diagnosis and management. This case emphasizes the importance of considering unusual etiologies in refractory sciatica cases and underscores the complexity of medical diagnosis. Increased awareness among healthcare providers can lead to improved patient outcomes and prevent diagnostic delays.

An unusual cause of inguinal swelling: Primary synovial osteochondromatosis.

Inguinal swelling should raise suspicion for hip joint osteochondromatosis. Early recognition, accurate diagnosis, and prompt surgical intervention are essential for optimizing patient outcomes.

Solitary bone plasmacytoma of the axis, a rare and challenging case with good outcomes.

INTRODUCTION: Plasmacytoma is a rare hematological malignancy with a more favorable prognosis than multiple myeloma. This case report focuses on a unique solitary bone plasmacytoma (SBP) at the craniovertebral junction (CVJ), managed through surgery and radiotherapy. CASE PRESENTATION: A 50-year-old patient presented with four months of worsening neck pain and cervicobrachial neuralgia. Despite treatment and physiotherapy, symptoms persisted. Clinical examination revealed neck stiffness, with no motor or sensory deficits. Radiographs and MRI showed an infiltrating axis lesion without instability signs. A CT-guided biopsy yielded inconclusive results. To address instability and establish a diagnosis, a curettage biopsy of the C2 posterior arch was performed, followed by occipito-C4 fusion using an iliac crest graft. Histological examination confirmed SBP. Adjuvant radiotherapy and chemotherapy were administered. At four-year follow-up, there was no multiple myeloma progression, but limited neck mobility were reported, with stable fusion observed on imaging. DISCUSSION: Solitary bone plasmacytoma primarily affects the axial skeleton, with rare upper cervical spine involvement. Diagnostic criteria include histological confirmation, normal bone marrow analysis, unremarkable imaging (except for the primary lesion), and absence of end-organ damage related to lymphoplasmacytic proliferative disorders. Clinical presentation is nonspecific, and MRI is valuable for soft tissue assessment. Radiotherapy is the primary treatment, with surgery reserved for specific indications. CONCLUSION: Solitary bone plasmacytoma is a rare condition with a favorable prognosis when promptly managed. This case underscores the importance of early diagnosis and treatment to prevent recurrence or multiple myeloma progression. A multidisciplinary approach, including surgery when necessary, is crucial for optimal outcomes.

One-stage combined approach en bloc vertebrectomy for primary Ewing's sarcoma of mobile spine in an adult patient: 3 years following aggressive surgery for a rare entity.

Key Clinical Message: Currently, there is no consensus on the optimal management of nonmetastatic Ewing's sarcoma of the mobile spine. However, associated to chemotherapy, aggressive surgery with en bloc wide resection seems to improve local control and survival. Abstract: Primary Ewing's sarcoma (EWS) of non-sacral spine is extremely rare, especially in middle-age. Therapeutic strategy aims: to large tumor resection, to provide spine stability and to avoid recurrence through chemo and radiotherapy. We report a case of thoracic spine EWS in an adult treated by combined approach en bloc vertebrectomy.

Conservative surgical management of surface osteosarcoma using 3D printing technology: An unusual case report and literature review.

INTRODUCTION AND IMPORTANCE: Surface osteosarcoma, a rare variant of osteosarcoma, poses unique challenges in diagnosis and treatment. This report discusses the application of 3D printing technology in the surgical management of a complex case involving a 27-year-old female patient with surface osteosarcoma in the proximal tibia. CASE PRESENTATION: A 27-year-old female patient presented with a progressively growing mass on her right knee, initially misdiagnosed as a benign bone tumor. Over five years, the lesion expanded from a well-corticated metaphyseal-epiphyseal outgrowth on the proximal tibia to involve the anterior tibial tuberosity. Radiological and histological evaluations confirmed well-differentiated paraosteal surface osteosarcoma. A multidisciplinary team opted for a conservative surgical approach, including resection of the anterior tibial tuberosity and patellar tendon. Precision was enhanced through 3D printing technology, which provided custom cutting guides. The reconstruction involved non-vascularized peroneus and iliac crest bone grafts. CLINICAL DISCUSSION: Accurate differentiation from benign lesions presents challenges. Achieving surgical resection with clear margins is pivotal for favourable outcomes, particularly in young patients. Chemotherapy yields limited benefits in low-grade surface osteosarcomas. Functional prognosis hinges on effective post-resection reconstruction. 3D printing technology facilitates meticulous surgical planning and guidance, enhancing the success of conservative surgical interventions. CONCLUSION: This case underscores the significance of a multidisciplinary approach, accurate diagnosis, and the integration of 3D printing technology in managing surface osteosarcomas. Conservative surgical resection, guided by precise planning and reconstruction, is critical for preserving functionality. Continued research and the adoption of innovative techniques hold promise for improving the quality of life and functional outcomes of individuals grappling with musculoskeletal tumors.

Primary giant hydatid cyst of the thigh: An unusual location.

Key Clinical Message: Primary hydatid cyst of the thigh is a rare condition requiring prompt recognition and accurate diagnosis for timely management and prevention of complications. Abstract: Muscular hydatid cysts are rare, with a frequency ranging from 1% to 5% even in endemic areas. The clinical presentation of muscular hydatidosis is typically subtle and lacks specific features, frequently resulting in delayed diagnosis. Herein, the authors describe a case of a primary hydatid cyst located in the thigh.

Giant cell tumor of the tendon sheath of the toe: case report of an unusual localization.

INTRODUCTION AND IMPORTANCE: The purpose of this study is to highlight a rare entity of unusual location and to report our therapeutic attitude in this regard, while detailing the diagnostic criteria and therapeutic management. CASE PRESENTATION: We describe the case of a 32 years old woman with a swelling of the big toe initially neglected by the patient, the evolution is marked by a discomfort when wearing shoes, it was a firm mass of 2 cm slightly painful without local inflammatory signs with bone lysis on radiography, The MRI showed an invasion of the flexor and extensor tendons of the hallux, our approach was a carcinological tumor exeresis and arthrodesis while preserving the toe since the pedicle was not invaded, the histological examination confirmed the diagnosis and the evolution is good without tumor recurrence. CLINICAL DISCUSSION: A giant cell tumor of the tendon sheaths (GCTTC) usually presents as a firm slow growing mass, the radiological assessment shows bone erosions, an MRI for extension assessment and histological confirmation are mandatory, malignant degenerations are unusual but should always be investigated, treatment is based on the most complete surgical resection to avoid the risk of recurrence, adjuvant radiotherapy can be done in case of incomplete resection but it is a controversial subject. CONCLUSION: GCTTC are benign tumors that may be locally malignant because of the invasion of noble structures, which makes their complete exeresis difficult. The diagnosis is based on clinical and radiological criteria and requires histological confirmation.

Bone metastases from renal cell carcinoma: 4 years after aggressive surgeries and anti-angiogenic therapy.

Management of bone metastases from renal cell carcinoma (RCC) has significantly changed after the era of targeted therapy that improved the overall survival (OS). Surgical decision-making remains a subject of controversy. We report a case of pelvic bone metastasis from RCC, 2 months after nephrectomy and surgery of a revealing clavicular metastasis.

Femoral nerve paralysis complicating a post-traumatic iliopsoas haematoma.

Paralysis of the femoral nerve secondary to iliopsoas haematoma is a rare post-traumatic complication. Because of the large differential diagnosis, a high level of suspicion is required for its early recognition. Treatment modalities are controversial due to the rarity of this entity. An 18-year-old student presented with complete paralysis of the knee extensors and a sensory deficit on the anterior side of the thigh 5 weeks after a sport accident. MRI of the lesser pelvis showed an iliopsoas haematoma. Surgical decompression was performed and recovery was complete at 6 months of follow-up.

[Spinal hydatidosis: diagnostic and treatment]. / Hydatidose vertébrale: approche diagnostique et thérapeutique.

Hydatidosis is a parasitic disease due to Echinococcus granulosus. Spinal hytatidosis is the most common form, characterized by high morbidity and mortality. It is often diagnosed only when neurological complications occur, due to the long clinical latency and the non specific nature of symptoms. Standard radiography may show lacunar, areolar abnormalities separated by irregular walls without clear boundaries. Magnetic resonance imagery can reveal hydatic abcess presenting typical liquid abcess and locoregional extension. Laboratory methods are of little assistance to the diagnosis. Complete surgical excision of the lesions remains the only curative treatment, in the same way as for malignant tumors. Surgical eradication of hydatic lesions is difficult, however. Indeed, the surgeon is guided by the macroscopic aspect, but the bone may be infiltrated by microvesicles without clear boundaries. It is now recommended to accompany surgery by medical treatment based on albendazole. Rapid diagnosis and treatment of spinal hyatidosis may yield long-term remission or even cure, especially in localized forms.

[Pott's disease of the upper cervical spine (three cases and literature review)]. / Mal de Pott du rachis cervical supérieur (à propos de 3 cas).

BACKGROUND: Sub-occipital Pott's disease is rare, but dangerous. AIM: To recall diagnostic aspects and spatially the neuroradiological ones of sub-occipital Pott's disease. METHODS: We describe three patients with sub-occipital Pott's disease followed in our department from 2002 to 2007. RESULTS: There were two women and one man (mean age at onset: 48 years). The early clinical picture was non specific. Exploration, in addition to standard radiographies included a computerized Tomography (CT) (n=2) and MRI (n=1 and n=3). The diagnosis was confirmed histologically on a biopsy of the abscess (n=1), a bacteriological evidence (n=2) and retained thanks to argument presumption (n=3). The treatment was based on quadruple antituberculous chemotherapy with external traction (n=2 and n=3) associated with urgent surgery (n=1) because of the worsening of the neurological complications. The evolution was fatal (n=1) and positive (n=2 and n=3). CONCLUSION: Tuberculosis should be suspected in patients with chronic torticollis and residing in an area when tuberculosis is endemic. Medical treatment is based on a quadruple antituberculosis chemotherapy with traction in the presence of atlanto-axial instability. Surgery is reserved in case of neurological worsening or persistent instability.

[Predictive criterions of unrecognized articular effraction after internal fixation of femoral neck fractures]. / Criteres predictifs d'une effraction articulaire meconnue apres osteosynthese d'une fracture du col du femur.

On the basis of a retrospective study comprising 91 cases of femoral neck fracture treated by DHS, unrecognized screw articular penetration was observed in 9 cases (9.9%). The coxa valga (Garden I) fractures are the most interested by this complication (7 cases out of 9). With a practical purpose and to avoid remarking each time complex measurements again we found that the distance "d" separating the end of the implant from subchondral bone calculated on antero posterior and lateral views and so the angle measured on the lateral view were two criteria significantly predictive of this technical error with respectively P: 0.005 and P: 0.0005. Thus during the surgical procedure taking these two measurements after the installation of the pin guide would allow attention on the risk incurred. In case of doubt, we have changed the way of this pin before resorting to drilling and tapping.