UNASSIGNED: Although not described more than a decade ago, fibro adipose vascular anomaly has grown of increasing importance as conventional management of arterio venous malformation with interventional radiology measures carry little success and cause significant morbidity in pediatric age groups like the case report we present here. Surgical resection, even though requiring significant loss of muscle bulk, is the mainstay of the treatment.
UNASSIGNED: An 11-year-old patient presented with equinus deformity and intensely tender calf and foot swellings in the right leg. Magnetic resonance imaging showed presence of two distinct lesions, one involving the gastrocnemius and soleus, and the other in the Achilles tendon for which en bloc resection of the tumor was performed. Histopathology of the specimens confirmed the diagnosis of fibro adipose venous anomaly.
UNASSIGNED: As per our knowledge, this is the first case of multiple fibro adipose venous anomaly confirmed by clinical features, radiology, and histopathology.

UNASSIGNED: Paratesticular fibrous pseudotumor (PFP) is a rare intrascrotal benign fibrous mass of uncertain aetiology, usually arising between testicular tunica layers and is supposed to be related to inflammatory reactive conditions. Because of morphological similarities to IgG4-related sclerosing fibro-inflammatory lesions, some authors recently postulated that PFP might belong to the IgG4-related disease (IgG4-RD) family. Considering the rarity of this lesion, only few cases have been reported in literature about the correlation between IgG4-RD and PFP. Management of PFP could be extremely challenging: due to the lack of typical clinical signs and the non-specific radiological characteristics, misapprehension does occur in the majority of cases, mainly because these intrascrotal mass may mimic testicular neoplasm, therefore leading to radical orchidectomy rather than a desirable testis-sparing surgery.
UNASSIGNED: Herein we report two cases of young males treated for PFP with histological feature of IgG4-RD. Patients underwent testicular sparing surgery. At 2-year follow-up no evidence of local or distant relapse nor testicular disorder was observed in both patients. An up-to-date review of the literature about the correlation between PFP and the IgG4-RD was carried out.
UNASSIGNED: PFP is an extremely rare condition with uncertain etiology being part of IgG4-RD family. Preoperative imaging mimics malignancy hence diagnosis is usually made by specimen analysis. Intraoperative frozen section is fundamental in order to guarantee conservative treatment that is feasible and safe after mid-term follow-up.

Solitary fibrous tumour of the pleura (SFT) is rare neoplasms and consist less than 5% of the primary tumours of the pleura. In the English literature, very few cases of giant solitary fibrous tumours have been described. We report a clinical case of an intrathoracic giant SFT of the pleura in a 62-year-old female patient. Additionally, we reviewed the clinical, imaging and histopathological features, the therapeutic management and the clinical course of giant SFTs published in the English literature. For this, we conducted a comprehensive electronic search at the PubMed using the key words giant, huge, big and enormous.

Tribbles 3 (TRIB3) modulates lipid and glucose metabolism, macrophage lipid uptake, with a gain-of-function variant associated with increased cardiovascular risk. Here we set out to examine the role of this pseudokinase in atherosclerotic plaque development. Human endarterectomy atherosclerotic tissue specimens analysed by immunofluorescence showed upregulated TRIB3 in unstable plaques and an enrichment in unstable regions of stable plaques. Atherosclerosis was induced in full body Trib3KO and Trib3WT littermate mice by injecting mPCSK9 expressing adeno-associated virus and western diet feeding for 12 weeks. Trib3KO mice showed expanded visceral adipose depot while circulatory lipid levels remained unaltered compared to wildtype mice. Trib3KO mice aortae showed a reduced plaque development and improved plaque stability, with increased fibrous cap thickness and collagen content, which was accompanied by increased macrophage content. Analysis of both mouse and human macrophages with reduced TRIB3 expression showed elongated morphology, increased actin expression and altered regulation of genes involved in extracellular matrix remodelling. In summary, TRIB3 controls plaque development and may be atherogenic in vivo. Loss of TRIB3 increases fibrous cap thickness via altered metalloproteinase expression in macrophages, thus inhibiting collagen and elastic fibre degradation, suggesting a role for TRIB3 in the formation of unstable plaques.

Paratesticular masses are not infrequent, however the diagnosis is challenging. Differentiation from testicular tumours is of utmost importance. One of the rare forms is a fibrous pseudotumours with only about a couple of hundred cases documented worldwide. We present a case of left paratesticular inflammatory pseudotumour.

UNASSIGNED: Sellar xanthogranulomas are rare intracranial lesions comprising <1% of all sellar lesions. They were described as a separate entity by the World Health Organization in 2000. Because of the paucity of sellar xanthogranuloma cases reported in the literature, they remain a diagnostic challenge with indefinite origin, clinical course, and outcome. The present study reports a case of sellar xanthogranuloma describing the clinical presentation, radiological/pathological characteristics, and outcome.
UNASSIGNED: A 43-year-old female, known to have diabetes, hypothyroidism, and polycystic ovarian syndrome, presented with a 2-week history of sudden right-sided facial deviation, periorbital pain, and moderate-intensity headache. The patient also reported amenorrhea not improving with polycystic ovarian syndrome treatment. Neurologic examination showed bilateral visual field defects and impaired visual acuity. Computed tomography scan, without contrast, revealed a hypodense sellar lesion with areas of hyperdensity. Magnetic resonance imaging showed a well-defined sellar lesion, exhibiting high signals on T1-weighted and T2-weighted images. The patient underwent microscopic trans-nasal trans-sphenoidal excision of the lesion. Histological sections of the sellar lesion revealed fibrous connective tissue with chronic inflammatory cells and cholesterol clefts, suggestive of xanthogranuloma. The patient is currently followed up at neurosurgery, endocrinology, and ophthalmology clinics with periodic laboratory/radiological investigations.
UNASSIGNED: Sellar xanthogranulomas remain rare intracranial lesions with few cases reported in the literature. Patients mostly present with severe hypopituitarism and visual dysfunction. They show no characteristic radiological features. The diagnosis is confirmed histopathologically, and the prognosis is generally favorable.

Congenital granular cell tumor (CGCT) is a rare benign lesion and presents as a fibrous mass arising from the alveolus in the newborn. The prenatal screening of lesions can help in parent counseling, determining the complications, as larger size lesions may interfere with normal delivery and require a cesarean section.

Angiomatoid fibrous histiocytoma (AFH) is a rare, slow-growing soft tissue tumor with an intermediate biologic potential and uncertain line of differentiation, and minimal metastatic potential. AFH may mimic both the clinical, histological, and radiological findings of several tumors; therefore, it is frequently misdiagnosed.  Three cases of AFH were included in this study. A six-year-old male and two females with an age of 12 and 17 years are presented. The primary locations were in the right shoulder (case 1), left medial proximal thigh (case 2), and left lateral knee (case 3). Two cases (cases 2&3) were presented with a painful mass. In the three patients, the masses were firm, mobile, and not attached to the skin.  Magnetic resonance imaging (MRI) was done, illustrating unspecific findings to reach a diagnosis. Needle biopsies were performed in all patient, and the diagnosis of AFH was reached. All three patients underwent wide surgical excision of the tumor. Patients were followed up routinely every three to four months with imaging studies to rule out recurrence and metastasis, for a period of 15 months (case 1), 26 months (case 2), and 19 months (case 3), which all resulted negatively.

Fibrous dysplasia of the spine in a polyostotic form is very rare, with fewer than 36 cases discussed in the literature and there is no such case in Indonesia that has been reported. The aim of this report is to present a case from Indonesia of polyostotic fibrous dysplasia isolated in the spine. We report a case of a 38-year-old Sundanese man with a 1-year history of progressive back pain and weakness of both lower extremities. There was no history leading to infection and no previous trauma. A physical examination revealed kyphoscoliotic deformity, a café au lait spot, tenderness at the thoracolumbar region, and neurological deficits. Laboratory studies were within normal ranges. Plain radiographs showed lytic lesion and kyphoscoliosis. Magnetic resonance imaging showed an endosteal scalloping, infiltrative process, expansion, and destruction in the vertebral bodies from T2 to L5. The findings of an aggressive destructive process was highly suspicious of a malignant process, relying on differential diagnosis and metastases, plasma cell myeloma, bone tumor and chronic infectious spondylitis. Histology revealed an irregularly oriented osteoid without osteoblastic rimming but surrounded by fibroblastic proliferation with a C-shaped sign. Investigations revealed a diagnosis of polyostotic fibrous dysplasia of the thoracolumbar spine in isolation. The patient underwent T5-S1 stabilization and bone grafting. At 1 year postoperative, the patient was asymptomatic; there was no recurrence and minimal neurological deficit with grade II on the modified McCormick scale. A case of the polyostotic form of fibrous dysplasia of the spine in isolation has never been reported in Indonesia. The extreme rarity of this type of presentation can pose a diagnostic dilemma, and in cases isolated to the spine, surgical treatment with posterior stabilization, decompression, and bone grafting gives a good functional outcome.

BACKGROUND: Intraparenchymal meningiomas without dural attachment are extremely rare, especially in female children. To our knowledge, fibrous intraparenchymal meningioma located in the temporal lobe has never been reported in female children. The significance in the differential diagnosis of lesions in the temporal lobe should be emphasized.
METHODS: A 12-year-old girl was admitted to our hospital, complaining of recurrent generalized seizures for 2 months. Magnetic resonance imaging demonstrated a solid lesion located in the temporal lobe. The lesion underwent gross total resection. Histopathological examination indicated that the lesion was a fibrous meningioma. Postoperative rehabilitation was uneventful.
CONCLUSIONS: This case report presents an extremely unusual intraparenchymal fibrous meningioma of the temporal lobe with peritumoral edema and reviewed 21 intraparenchymal meningioma cases in children and to discuss the clinical presentation and treatment, differential diagnosis, and radiological features.