Giant orbital leiomyoma in a pediatric patient: diagnostic and therapeutic challenge.

This manuscript describes an exceptional case of a long-standing orbital leiomyoma in a 14-year-old male. The tumor was unusually large, causing severe proptosis and significant involvement of the ocular muscles. The patient presented with amaurosis, complete ophthalmoplegia, spontaneous eye pain, and the inability to close the eyelids, leading to psychological distress. Due to the tumor's size and progression, a right orbital exenteration was performed to remove all orbital contents, including the tumor and the eyeball. The surgical procedure aimed to prevent tumor recurrence and improve the patient's quality of life. The histopathological analysis confirmed the diagnosis of orbital leiomyoma. This case presents a particular interest due to the degree of evolution it has reached. Complete tumor excision and long-term follow-up are necessary to prevent recurrence and ensure optimal patient outcomes. This report underscores global healthcare disparities and the complexity of managing rare orbital neoplasms in diverse country settings.

Risk factors associated with surgical site infection in patients with musculoskeletal cancer / Fatores de risco associados à infecção de sítio cirúrgico em portadores de câncer musculoesquelético / Factores de riesgo asociados a la infección del sitio quirúrgico en pacientes con cáncer musculoesquelético

Background and objectives: surgical site infections (SSI) continue to be a major concern in orthopedic oncology and pose as great a challenge as cancer recurrence, despite the preventive potential of surgery. SSI can be avoided if evidence-based measures are taken. The objective was to assess the frequency of infections in oncological orthopedic surgeries and associated risk factors and inflammatory markers in a reference hospital in the state of Pernambuco. Methods: the frequency of SSI, the identification of isolated microorganisms, the risk factors and the profile of Th1 and Th2 inflammatory markers (IL-2, IL-4, IL-6, IL-10, TNF and IFN-Ƴ) in patients with musculoskeletal cancer were analyzed. Results: SSI were found in 9.1% of patients undergoing orthopedic surgery. Bivariate analysis revealed that a surgical team comprising more than five members (p=0.041) and the need for intraoperative transfusion (p=0.012) were correlated with a higher risk of SSI. The measurement of ultrasensitive C-reactive protein levels to assess the inflammatory response after SSI showed results that were superior to the reference values for each sample, ranging from >5 to >200mg/dl by the immunoturbidimetric method. Of the IL-2, INFγ and TNF (Th1) and IL-4, IL-6, IL-10 (Th2) levels, only interleukin 6 showed high levels, between 6.68 and 58.76 pg/mL. Conclusion: the study found that surgical team with five or more members and blood transfusion were factors associated with the development of SSI in orthopedic surgery in patients with musculoskeletal cancer. Among the inflammatory markers, interleukin 6 (IL-6) showed the highest correlation with the outcome.(AU)

Justificativa e objetivos: as infecções do sítio cirúrgico (ISC) continuam sendo uma grande preocupação na oncologia ortopédica e representam um desafio tão grande quanto a recorrência do câncer, apesar do potencial preventivo da cirurgia. As ISC podem ser evitadas se forem tomadas medidas baseadas em evidências. O objetivo foi avaliar a frequência de infecções em cirurgias ortopédicas oncológicas e os fatores de risco e marcadores inflamatórios associados em um hospital de referência no estado de Pernambuco. Métodos: foram analisados a frequência de ISC, a identificação de microrganismos isolados, os fatores de risco e o perfil de marcadores inflamatórios Th1 e Th2 (IL-2, IL-4, IL-6, IL-10, TNF e IFN-Ƴ) em pacientes portadores de câncer musculoesquelético. Resultados: as ISC foram encontradas em 9,1% dos pacientes submetidos à cirurgia ortopédica. A análise bivariada revelou que uma equipe cirúrgica composta por mais de cinco membros (p=0,041) e a necessidade de transfusão intraoperatória (p=0,012) foram correlacionadas com maior risco de ISC. A dosagem dos níveis de proteína C reativa ultrassensível para avaliação da resposta inflamatória após ISC apresentou resultados superiores aos valores de referência para cada amostra, variando de >5 a >200mg/dl pelo método imunoturbidimétrico. Dos níveis de IL-2, INFγ e TNF (Th1) e IL-4, IL-6, IL-10 (Th2), apenas a interleucina 6 apresentou níveis elevados, entre 6,68 e 58,76 pg/mL. Conclusão: o estudo constatou que equipe cirúrgica com cinco ou mais membros e transfusão sanguínea foram fatores associados ao desenvolvimento de ISC em cirurgia ortopédica em pacientes com câncer musculoesquelético. Entre os marcadores inflamatórios, interleucina 6 (IL-6) apresentou maior correlação com o desfecho.(AU)

Justificación y objetivos: las infecciones del sitio quirúrgico (ISQ) siguen siendo una preocupación importante en la oncología ortopédica y representan un desafío tan grande como la recurrencia del cáncer, a pesar del potencial preventivo de la cirugía. Las ISQ se pueden prevenir si se toman medidas basadas en la evidencia. El objetivo fue evaluar la frecuencia de infecciones en cirugías ortopédicas oncológicas y los factores de riesgo y marcadores inflamatorios asociados en un hospital de referencia en el estado de Pernambuco. Métodos: se analizaron la frecuencia de ISQ, la identificación de microorganismos aislados, los factores de riesgo y el perfil de marcadores inflamatorios Th1 y Th2 (IL-2, IL-4, IL-6, IL-10, TNF e IFN-Ƴ) en pacientes con cáncer musculoesquelético. Resultados: se encontraron ISQ en el 9,1% de los pacientes sometidos a cirugía ortopédica. El análisis bivariado reveló que un equipo quirúrgico compuesto por más de cinco miembros (p=0,041) y la necesidad de transfusión intraoperatoria (p=0,012) se correlacionaron con un mayor riesgo de ISQ. La medición de los niveles de proteína C reactiva ultrasensible para evaluar la respuesta inflamatoria después de la ISQ presentó resultados superiores a los valores de referencia para cada muestra, variando de >5 a >200 mg/dl por el método inmunoturbidimétrico. De los niveles de IL-2, INFγ y TNF (Th1) e IL-4, IL-6, IL-10 (Th2), solo la interleucina 6 mostró niveles elevados, entre 6,68 y 58,76 pg/mL. Conclusión: el estudio encontró que el equipo quirúrgico con cinco o más miembros y la transfusión el estudio encontró que un equipo quirúrgico con cinco o más miembros y transfusión de sangre fueron factores asociados con el desarrollo de ISQ en cirugía ortopédica en pacientes con cáncer musculoesquelético. Entre los marcadores inflamatorios, la interleucina 6 (IL-6) mostró la mayor correlación con el resultado.(AU)

An unusual extranodal natural killer/T-cell lymphoma presenting as chronic laryngitis: A case report.

RATIONALE: Nasal-type, extranodal natural killer (NK)/T-cell lymphoma is a rare lymphoma. The tumor usually shows ulcerative and necrotic lesions in the nasal cavities and sinuses. Tissue involvement outside the nasal cavity is uncommon. PATIENT CONCERN: We describe a 30-year-old man with a 2-month history of hoarseness, weight loss, and dyspnea. DIAGNOSIS: Magnetic resonance image (MRI) showed edema of the larynx with obliteration of the airway. Laryngoscopic examination described necrotic tissue in the glottis and larynx. The biopsy showed chronic, necrotizing laryngitis, with no granulomas, vasculitis, or atypical cells. The immunologic and microbiologic study was negative. Later, after immunosuppressive therapy, the patient presented erythema and diffuse enlargement of the right arm. MRI showed myositis of the biceps and brachial muscles. Infection was rule out, and direct microscopy showed an extensive muscle infiltration by mononuclear cells and abundant mitosis. Immunohistochemistry was positive for CD3, CD8, Ki 67 (90%), and CD56 compatible with extranodal NK/T cell lymphoma. INTERVENTIONS: The patient initially received immunosuppression treatments (corticoids, cyclofosfamide, and Rituximab) with relapsing episodes. When lymphoma was diagnosed, chemotherapy was started. OUTCOMES: The patient died during chemotherapy. LESSONS: Nasal-type, extranodal NK/T-cell lymphoma should be suspected even when there are no classical findings of neoplasms on histology. Immunohistochemistry is mandatory to rule it out.

[Abdominal wall repair after total resection of desmoid fibroma in a patient with diabetes mellitus type 1]. / Rekonstruktsiya perednei bryushnoi stenki posle radikal'noi rezektsii desmoidnoi fibromy na fone sakharnogo diabeta 1-go tipa.

Desmoid fibroma (DF) is a rare connective tissue tumor comprising about 0.03-0.13% of all neoplasms. DF has a low potential for malignant transformation, but it is characterized by aggressive course and unfavorable prognosis. The main contingent of patients consists of women of reproductive age. Despite the nearly two-century history of study, there are certain unsolved problems including endocrine problems associated with this disease. We report a 30-year-old female with DF and diabetes mellitus type 1. Total resection of the affected right rectus abdominis muscle was performed in a single block with aponeurotic sheath and peritoneum after normalization of carbohydrate metabolism. Muscular aponeurotic defect 27�10 cm was closed after separation of abdominal wall structures and implantation of polypropylene prosthesis. There are no X-ray and clinical signs of DF recurrence or postoperative hernia after 8 months.

Anaplastic lymphoma receptor tyrosine kinase-negative inflammatory myofibroblastic tumor of triceps brachii: Case report.

Inflammatory myofibroblastic tumor (IMT) is a non-neoplastic benign lesion comprising various inflammatory cells, including myofibroblasts and vascular tissues. It is a rare tumor that sometimes shows similar signs and progression as malignant tumors. The anatomical sites of IMTs include the lungs, liver, orbit, skin, mesentery, and maxillary sinus, but they rarely occur in the limb musculoskeletal system. To our knowledge, no case of neurological symptoms caused by the tumor in the triceps brachii muscle has been reported. In this article, we report the case of a 42-year-old male patient with an IMT that grew rapidly in the triceps brachii muscle and consequently caused symptoms of ulnar nerve lesion owing to its increasing size. The patient showed no ulnar nerve lesion symptoms after undergoing wide excision and was diagnosed with anaplastic lymphoma receptor tyrosine kinase- negative IMT.

Papillary thyroid carcinoma arising in ectopic thyroid tissue within sternocleidomastoid muscle: a review of current literature.

The prevalence of ectopic thyroid tissue as consequence of an aberrant migration of thyroid during embryogenesis ranges up to 10% in autopsy studies. The differential diagnosis between the relatively rare occurrence of a primary carcinoma arising in ectopic thyroid tissue and the more frequent presence of cervical lymph node metastasis from papillary thyroid carcinoma (PTC) might represent a difficult challenge in the clinical practice. The clinical relevance of these lesions lies in their risk of hidden primary thyroid cancer. Our intention is to provide in this review the current limited data available and to report an unusual localization of primary PTC arising from an extra-thyroid area, responsible for a solitary cervical mass as initial manifestation. The tumor developed in an ectopic thyroid tissue embedded within the clavicular head of the sternocleidomastoid muscle and was completely separated from the thyroid. Surgical excision of ectopic thyroid tissue with clavicular head of sternocleidomastoid muscle along with total thyroidectomy and central and selective lateral neck dissection were carried out. Histopathology was diagnostic for ectopic PTC and no primary lesions in the thyroid gland neither metastatic lymph nodes were found. Tumor cells were positive for thyroid transcription factor-1and thyroglobulin, and negative for CD56. A postoperative adjuvant radioiodine ablation was given after recombinant human thyroid-stimulating hormone (TSH) stimulation and the post-treatment whole body scan was negative. After the evaluation at six months showing negative neck ultrasound and undetectable thyroglobulin levels, while TSH suppressed and after recombinant human TSH stimulation, the patient was re-evaluated every six months. At two years, the patient remained completely free of disease and is currently on substitutive dose of l-thyroxine. Endocrinologists and neck surgeons must be aware of the rare possibility of primary PTC arising from ectopic thyroid tissue within the sternocleidomastoid muscle.

Intraneural hemangioma in Klippel-Trenaunay syndrome: role of musculo-skeletal ultrasound in diagnosis-case report and review of the literature.

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder with an incidence of 1 in 100,000. It is characterized by a triad of capillary malformations (hemangiomas) or port-wine stains, venous varicosities, and bony- or soft-tissue hypertrophy. The capillary malformation is usually confined to a single extremity, usually a lower limb. The disease can lead to various morbidities, such as bleeding, deep vein thrombosis, venous ulcers, and embolic complications. We report a case of an 11-year-old girl who presented with the three classical symptoms of KTS, with port-wine stains in the left leg, an enlarged and elongated left leg, and soft-tissue hypertrophy and multiple venous varicosities in the left tibia. A subcutaneous hemangioma along with intramuscular hemangiomas in the leg muscles was noted with increased adipose tissue. The rare finding of an intraneural hemangioma of the distal posterior tibial nerve was also diagnosed. Ultrasound of the lower limb was the main tool in making the diagnosis of KTS. X-Ray and MRI were ancillary imaging modalities. This article describes the case study of the child and the findings of a detailed ultrasound examination.

Exoftalmos unilateral secundario a metástasis en músculo recto interno de adenocarcinoma de esófago / Unilateral exophthalmos secondary to esophageal adenocarcinoma metastasis to the medial rectus muscle

Varón de 51 años con diagnóstico de adenocarcinoma de esófago estadio IV. Es referido desde oncología médica para valoración oftalmológica urgente por llamativo exoftalmos izquierdo de dos semanas de evolución además de diplopía binocular. En la exploración oftalmológica destaca la presencia de un exoftalmos axial no reductible y una limitación de la motilidad ocular a la levoducción. Se realiza una tomografía axial computarizada craneal urgente donde se objetiva una masa orbitaria izquierda polilobulada y heterogénea, intra- y extraconal, que engloba al músculo recto interno, compatible con metástasis, además de otra pequeña masa extraconal en la base de la órbita contralateral. Se indica entonces tratamiento con radioterapia paliativa. Las metástasis en los músculos extraoculares son un hallazgo muy poco frecuente, pero deben sospecharse ante un caso de exoftalmos unilateral y, de ser necesario, remitir al paciente para realizar las pruebas complementarias correspondientes

A case is presented on a 51 year-old man with stage IV oesophageal adenocarcinoma. The patient was referred by the Oncology Department urgently for a same-day assessment by an ophthalmologist due to two weeks of severe unilateral exophthalmos and binocular diplopia. A comprehensive eye exam revealed the presence of an axial non-reductive exophthalmos and a limitation in left eye levoduction. A computed tomography scan was performed that showed a multiple lobed, intra-and extra-conal, heterogeneous left orbital mass, that surrounded the internal rectus muscle, compatible with metastasis, as well as another small extraconal mass at the base of the contralateral orbit. Palliative radiotherapy was then indicated. Metastases in the extraocular muscles are a very rare finding, but should be suspected in a case of unilateral exophthalmos and, if necessary, refer the patient to have the corresponding complementary tests performed

Edema palpebral unilateral secundario a demodicosis granulomatosa / Unilateral palpebral oedema secondary to granulomatous demodicosis

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Unilateral exophthalmos secondary to esophageal adenocarcinoma metastasis to the medial rectus muscle. / Exoftalmos unilateral secundario a metástasis en músculo recto interno de adenocarcinoma de esófago.

A case is presented on a 51 year-old man with stage IV oesophageal adenocarcinoma. The patient was referred by the Oncology Department urgently for a same-day assessment by an ophthalmologist due to two weeks of severe unilateral exophthalmos and binocular diplopia. A comprehensive eye exam revealed the presence of an axial non-reductive exophthalmos and a limitation in left eye levoduction. A computed tomography scan was performed that showed a multiple lobed, intra-and extra-conal, heterogeneous left orbital mass, that surrounded the internal rectus muscle, compatible with metastasis, as well as another small extraconal mass at the base of the contralateral orbit. Palliative radiotherapy was then indicated. Metastases in the extraocular muscles are a very rare finding, but should be suspected in a case of unilateral exophthalmos and, if necessary, refer the patient to have the corresponding complementary tests performed.

Anti-Amphiphysin-associated limbic encephalitis in a 72-year-old patient with aortic angiosarcoma.

Paraneoplastic autoimmune encephalopathic syndromes have been described most often in association with small cell lung cancer or breast cancer, tumours of the ovaries, testes, lymphoma and thymoma. Antibodies associated with paraneoplastic encephalopathies are, among others, anti-Hu, anti-Ma2 and, in part, anti-N-methyl-D-aspartate(NMDA)-receptor antibodies. Here, we present the case of a 72-year-old patient hospitalised due to progressive cognitive decline and disorientation. Diagnostic workup revealed paraneoplastic anti-amphiphysin associated limbic encephalitis on the basis of an aortic angiosarcoma with metastases to kidney, muscle and bones. Highly aggressive chemotherapy as well as immunosuppressive therapy and cytoreductive laparoscopic nephrectomy were initiated. However, follow-up revealed further tumour progress and a worsening of neurological symptoms.

Intramuscular hemangioma within the biceps brachii causing the limitations of elbow extension and forearm pronation: A case report.

RATIONALE: Intramuscular hemangiomas are rare benign vascular neoplasms, merely accounting for 0.8% of all hemangiomas. Moreover, there are few case reports of intramuscular hemangiomas in the upper extremities. PATIENT CONCERNS: A 24-year-old male patient presented with a 5-year history of intermittent pain of the right elbow joint. He had observed a swelling of the right cubital fossa over the past 2 years, leading to the limitations of elbow extension and forearm pronation. DIAGNOSIS: The patient was diagnosed with intramuscular hemangioma of the biceps brachii. INTERVENTIONS: Surgical excision of the tumor was performed for this patient and postoperative early functional exercises were permitted. OUTCOMES: The movements of the right elbow and forearm reached the normal range of motion at 5 weeks after surgery. There was no evidence of recurrence during the 5-month follow-up. LESSONS: Optimal management of intramuscular hemangioma is critical, including precise evaluation, good microsurgical technique and early functional exercises, which may result in a satisfying outcome.

Mazabraud's Syndrome: A Case Report and Up-To-Date Literature Review.

OBJECTIVE: Mazabraud's syndrome is a rare form of bone fibrous dysplasia associated with intramuscular myxomas. Fibrous dysplasia, is generally localized to pelvis and femur and it results in a fragile bone with deformities, pain, pathological fractures and functional impairment. Intramuscular myxomas, are rare benign mesenchymal neoplasms that exceptionally may evolve to malignant forms. METHODS: This case report describes a 66-year-old woman with Mazabraud's Syndrome (MS), characterized both by monostotic right femur fibrous dysplasia and by a solitary intramuscular myxoma at the right quadriceps muscle, that underwent a long-term treatment (4 years) with intravenous zoledronic acid. RESULTS: Zoledronic acid therapy rapidly lowered bone pain together with a reduction of intramuscular myxoma volume, but did not affect the extension of fibrous dysplasia. No adverse effects have been observed during treatment. CONCLUSION: Highly active bisphosphonates are commonly used for the treatment of bone metabolic disorders and they are generally well tolerated. Zoledronic acid may represent a promising alternative to surgical intervention in MS, although its use in rare form of bone fibrous dysplasias is still controversial.