Xanthomatous Changes Research Articles

Extensive Xanthomatous Change in an Ovarian Serous Borderline Tumor: More Than Just a Mere Coincidence

AbstractSerous borderline tumors of the ovary fall in between the benign and malignant category. These tumors are often diagnosed early, and the prognosis is usually favorable. However, there are variations in the histopathological findings of borderline ovarian tumors. Xanthomatous change is one such rare change in which deposition of lipid-rich material is seen morphologically as xanthoma cells within tumor tissue and reports of this are sparse. There is postulated literature on xanthomatous change being a contributing factor for progression of cancer. We present a case of a 75-year-old postmenopausal female with complaint of pain abdomen since 3 days, diagnosed radiologically as right ovarian mucinous cystadenoma/cystadenocarcinoma. Histopathology revealed a serous borderline tumor with extensive xanthomatous change. This case presents important thoughts on exploring the connection of xanthomatous change with borderline ovarian tumors, which till date is a matter of debate.

Comprehensive study of ancient schwannoma: Exploring histomorphological diversity and diagnostic challenges.

Background: Ancient schwannoma is a rare subtype of schwannoma characterized by an extended duration of development and distinctive degenerative changes. These changes encompass relative loss of Antoni type A areas, perivascular hyalinization, hemorrhage, cystic necrosis, calcification, and the presence of atypical nuclei that can mimic sarcomatous pleomorphism. These histologic features often lead to diagnostic challenges, with ancient schwannomas frequently being misdiagnosed as malignant tumors. Objectives: This study aims to provide a comprehensive evaluation of the histomorphological spectrum observed in ancient schwannoma. Methods: A retrospective analysis was conducted on 248 schwannoma cases received at our tertiary health centre, spanning the years 2017 to 2023. Among these cases, 25 were identified as ancient schwannoma. Extensive examination of degenerative changes was performed using hematoxylin and eosin-stained paraffin-embedded tissue sections under light microscopy. Results: Patient ages ranged from 22 to 82years, with a nearly equal distribution between genders (12 females and 13 males). Tumors were located in various anatomical sites including the forearm, brain, abdomen, retroperitoneum, intradural space, lumbar region, and pelvis. Tumor dimensions varied from 1.5 to 11cm. Histologically, most cases exhibited nuclear atypia, cystic changes, hemorrhage, and siderophages, along with perivascular hyalinization, myxoid change, calcification, and xanthomatous change. Immunohistochemistry confirmed the neural origin of these tumors. Conclusion: Recognition of the diverse spectrum of secondary changes, coupled with the presence of focal areas showing paucicellular and cellular spindle cell arrangements, is crucial for the accurate diagnosis of ancient schwannoma. This study underscores the importance of histomorphological evaluation in distinguishing these benign tumors from malignant counterparts, thereby guiding appropriate clinical management strategies.

Perifollicular Xanthoma Occurring in Patients after Erlotinib Treatment.

Erlotinib (Tarceva®) is a drug used for the treatment of non-small cell lung cancer in patients with epidermal growth factor receptor (EGFR) mutations. Since EGFR is expressed in the skin, EGFR inhibitors commonly develop cutaneous side effects such as acneiform eruption, paronychia, telangiectasias, and abnormal eyelash growth. A 56-year-old man and a 46-year-old man visited the dermatology clinic complaining of yellowish papuloplaques and acneiform eruption on the face. They had been treated with erlotinib for lung cancer. Since patients using EGFR inhibitors are increasing, physicians should be aware that xanthomatous change can occur in severe acneiform eruptions after erlotinib treatment.

Xanthomatous meningioma, a rare histological variant: Case report

Xanthomatous meningioma is a WHO grade I metaplastic meningioma where neoplastic cells contain lipid-filled vacuolated cytoplasm. The origin of xanthomatous meningiomas is believed to be from meningothelial cells but diagnosis remains difficult because of their close resemblance with the histiocytes. Peculiar radiographic features may aid in diagnosis, however, definitive diagnosis requires immunohistochemical staining. We report a case of 43-year-old male with sphenoid wing meningioma revealing xanthomatous changes and received treatment as grade 1 meningioma. Though the exact pathophysiology remains unknown, we believe focal or gross metaplastic changes lead to transformation of tumor cells into the xanthomatous subtype. Authors discuss this interesting a rare histologic variant with discussion of the relevant literature.

Recurrent Schowannoma With Extensive Xanthomatous Changes: A Case Report

Schwannoma is one of the most common benign intracranial tumors, which originates from the Schwann cells of neural structures in the upper part of the vestibular branch of the eighth cranial nerve. This report describes a 64-year-old woman with headache and vertigo who had a past surgical history of acoustic schwannoma about 10 years ago. The patient underwent excisional surgery. Histological examinations revealed schwannoma with extensive xanthomatous changes. Immunohistochemistry staining confirmed the diagnosis. Base on the diagnosis, the patient received no more treatment. After 6 months of close follow-up examinations, no recurrence and complication were observed. Schwannoma could be seen with a vast variety of histologic changes. Recurrence of Schownnoma is uncommon in patients without neurofibromatosis, but it should be considered in a proper clinical context.

Malignant Nerve Sheath Tumor with Secondary Intradural Extramedullary Metastases

We present a case of a 65 year old male patient of a malignant nerve sheath tumor with multiple intradural, extra medullary metastases and its preoperative imaging on MRI. Patient presented with complaints of pricking type of pain the cervical and dorsolumbar region since one year which was progressive since the day of onset of the symptoms. Introduction Nerve sheath tumors account for 80 to 90 % of all intradural extramedullary neoplasms 1 . Two main types of nerve sheath tumors are found in the spine: schwannoma (also known as neurinoma or neurilemoma) and neurofibroma. Schwannomas are lobulated, grossly encapsulated, well circumscribed round or oval tumors that often show cystic degeneration, hemorrhage and xanthomatous changes. Schwannomas arise eccentrically from their parent nerve; primary malignant peripheral nerve sheath tumors occur but are very rare 2 . Schwannomasal most never become malignant whereas sarcomatous transformation occurs in 4% to 11%of patients with neurofibromatosis 3. Case Presentation A 65 year old male patient presented to outpatient department of orthopedic complaining of a pricking type of pain in the cervical and dorso lumbar region since one year and was progressive since the day of onset of the symptoms. Physical examination demonstrated loss of patient strength of the right arm with restriction of movements. The patient was referred to the department of Radio-diagnosis for imaging workup, An MRI Study of the cervical spine was requested, imaging was conducted using 1.5 Tesla Siemens MR Machine. Axial GRE, sagittalT1W and T2W sequences were taken at the level of the cervical spine with screening of the brain and whole spine. MRI of the cervical spine revealed two intradural extramedullary lesions, the larger lesion was well defined which was present at the level of the foramen magnum, closely adherent to the brain stem. The lesion was indenting cerebellar tonsils with outaltered signal intensities in the basion, ophisthion and the posterior elements. Another lesion was noticed at the level of C6 & C7 vertebral bodies, the lesion was well defined and www.jmscr.igmpublication.org Impact Factor 5.244 Index Copernicus Value: 5.88 ISSN (e)-2347-176x ISSN (p) 2455-0450 DOI: http://dx.doi.org/10.18535/jmscr/v4i4.13

Primary dural hemangiopericytoma with xanthomatous change mimicking meningioma: a case report with brief review of literature

Hemangiopericytomas of the Central Nervous system present as a distinct clinicopathologic entity, having been categorized as a “mesenchymal-meningothelial tumour”, according to the current WHO classification. Not only is the lesion rare, but it also bears imaging, macro-micro and immunohistochemical similarities to the commoner tumour-meningioma and to the solitary fibrous tumour and has an indolent biological behaviour unlike the other two. We present a case of a 48 year old lady, diagnosed on imaging as meningioma, which turned out to be a dural hemangiopericytoma after complete histological and immunohistochemical work up. Excluding hemangiopericytoma in lesions presenting as intra-cranial tumours is critical, as the tumour is known for recurrence and metastasis, thereby warranting more aggressive treatment by the neurosurgeons, aiding in better patient care. We report this case not only because of its rarity, but also to show the xanthomatous change the tumour showed, not documented in literature so far.Bangladesh Journal of Medical Science Vol.14(2) 2015 p.206-209

A case of multicentric reticulohistiocytosis

Fig. 2 e ‘Coral bead’ appearance at the nail beds. A 32 year old lady with no past commorbities presented with inflammatory deforming polyarticular arthritis of small and large joints, upper and lower limbs, symmetrically for last 2 years. She also had developed nodular and papular skin lesions simultaneously with the onset of the joint pains with low grade fever andweight loss of 10 kg in 2 years. Shehadactive synovitis with swollen and tender knees, elbows, metacarpophalangeal joints. She had ‘Piano hand’ deformities of hands (Fig. 1). Skin colouredpapulonodular lesionswereseenover extensor surface of forearms, knees, elbows, knuckle, proximal interphalangeal jointsofhandandpinna.The lesionsrangedfrom1 to5cm.Over the knees the nodules coalesced to give a ‘cobble stone appearance’. At the nail beds there was clustering of the small papules giving to the characteristic ‘coral bead’ appearance (Fig. 2). X-ray both handswithwrists showed a severe erosive arthritis (Fig. 3). Biopsy from her nodular skin lesions showed focal papillomatosis in the dermis and well circumscribed nodular zone comprised of sheets of histiocytes with epitheloid and xanthomatous change. Majority of histiocytes had abundant granular eosinophilic cytoplasm (oncocytic histiocytes) consistent with reticulohistiocytosis. Patient was put on treatment with methotrexate15mg/week þ lefunomide 10 mg/day.

Clinicopathological features of cerebral lipoastrocytoma

Objective To explore the clinicopathological features, immune phenotype, diagnosis and differential diagnosis, treatment and prognosis of cerebral lipoastrocytoma. Methods Retrospective analysis of the clinical manifestations, histopathological and immunohistochemical features were conducted in one case of cerebral lipoastrocytoma. Results A 48-year-old male presented with numbness and inflexibility of the fourth and little fingers of his left hand over the previous 2 weeks. Cranial MRI revealed a space-occupying lesion with cystic degeneration in the right parietal lobe that showed obvious enhancement after contrast administration. The patient subsequently underwent craniotomy with stereotactic gross total excision of the lesion. Adjuvant chemotherapy and radiation therapy were not administered. Histologically the tumor showed classical features of low-grade astrocytoma, including a few scattered medium-large neuron-like cells with prominent nucleoli and abundant cytoplasm. Most notably, the glial cells contained fat droplets or vacuoles giving an appearance of mature adipocytes. Focally microcystic change was evident resulting from adipocyte-like cells fusion with each other. Immunohistochemically, the tumor cells were reactive for glial fibrillary acidic protein (GFAP) and S-100 protein (S-100), focally positive for WT-1, weakly positive for oligodendrocytes transcription factor-2 (Olig-2), and negative for isocitrate dehydrogenase 1 (IDH1). Meanwhile, the tumor cells also expressed several neuronal markers including synaptophysin (Syn), microtube-associated protein-2 (MAP-2), neurofilament (NF), neuron specific enolase (NSE) and CD34. P53 protein was weakly expressed in 5% of tumor cells. Ki-67 labeling index was low (1% ). The patient remained well without recurrence 20 months after surgery. Conclusions Cerebral lipoastrocytoma is an extremely rare tumor. Histologically, the tumor showed classical features of low-grade astrocytoma and extensive lipomatous differentiation. Interestingly, the present case also showed neuronal differentiation confirmed by positive staining with neuronal markers. Diagnosis needs to be combined with pathological morphology and immune phenotype, which can distinguish from other neuroepithelial tumors with fat cell differentiation or xanthomatous change. doi：10.3969/j.issn.1672-6731.2014.01.009

A Purely Cystic Giant Sacral Schwannoma Mimicking a Bone Cyst: A Case Report

Approximately 1--5% of the spinal schwannomas arise in the sacrum and frequently grow to a considerable size before becoming symptomatic; hence, the term “giant sacral schwannoma” refers to tumors extending into the vertebral body and the extraspinal space (1). Most schwannomas are solid or heterogeneous solid tumors, but they can rarely undergo cystic degeneration, xanthomatous change, or hemorrhage (2). A completely cystic appearance with only thin peripheral wall enhancement is extremely rare. A few cases of solitary cystic schwannomas arising from the spine have been previously reported (3-8). To the best of our knowledge, there is only one previous reported case of a giant solitary cystic schwannoma of the sacrum. Here, we describe a rare case of a totally cystic giant sacral schwannoma presenting as lumbar back pain. Institutional review board approval was obtained.

Meningothelial meningioma in a Malayan sun bear (Helarctos malayanus)

A 24-year-old, spayed female Malayan sun bear (Helarctos malayanus) in the Taipei Zoo (Taipei, Taiwan) showed clinical signs of slowly progressive anorexia, dullness, compulsive pacing, and circling. The animal subsequently developed acute severe stupor and persistent recumbency. Postcontrast study of computed tomography revealed a spheroid, extra-axial mass with strong but heterogeneous hyperattenuation in the left temporal lobe of the cerebrum. At necropsy, a solitary, well-circumscribed intracranial mass measuring 3 cm × 2.5 cm × 2 cm was attached to the left pyriform lobe with compression of the adjacent neuroparenchyma. Cytological examination obtained from the mass revealed large clumps and sheets of cohesive polyhedral cells with round nuclei, wispy cytoplasm, and indistinct cell borders. Microscopically, the mass was composed of densely packed round to polygonal cells arranged in lobules and small nests. Psammoma bodies, xanthomatous change, and cholesterol deposition were also noted. Immunohistochemical staining of the tumor was positive for vimentin, pancytokeratin, cytokeratin (CK)34BE12, neuron-specific enolase, and epithelial membrane antigen, but negative for glial fibrillary acidic protein and S100 protein. The cytological, histological, and immunohistochemical features were compatible with a meningothelial meningioma.

Synchronous multicentric pleomorphic xanthoastrocytoma with anaplastic features

Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumor with a relatively favorable prognosis. However, PXA cases having several recurrent patterns with poor prognosis have been reported in recent years, and a new concept of PXA with anaplastic features has been proposed. The present case was of a 55-year-old male who presented with weakness and numbness of right upper and lower limbs since 3 months along with difficulty in walking as well as difficulty in speaking since then. He also complained of headache since 9 months. The magnetic resonance imaging study revealed two nodular, homogeneously enhancing lesions, approximately 1 cm in size in the right cerebral hemisphere. Clinical and radiological examinations were suggestive of a metastatic neoplasm. A right frontal craniotomy was performed for excisional biopsy of both lesions. Histopathological findings showed that the tumor was PXA with strong pleomorphism, xanthomatous changes, extensive areas of tumor necrosis, and increased mitotic activity. From these findings, the histopathological diagnosis PXA with anaplastic features was given. Synchronous multicentric PXA presents unique challenges in that gross total resection would impose significant surgical morbidity; histological homogeneity among the lesions cannot be confirmed; and the well-described potential for anaplastic transformation may be increased with multiple lesions. The optimal treatment for patients with this rare and challenging diagnosis awaits further study.

Sarcomatoid transformation of a pleomorphic xanthoastrocytoma

Pleomorphic xanthoastrocytoma (PXA) is an uncommon, superficially situated astrocytic tumour typically occurring in children and young adults and characterised by cellular pleomorphism and frequent xanthomatous change. Classic forms of PXA are WHO Grade II but not all PXA follow a ‘benign' course. Recurrence is common, occurring in 72% of patients within 5 years. Overall survival is estimated at 81% at 5 years and 70% at 10 years. An estimated 15– 20% of PXA demonstrate anaplastic features and around 10% develop overtly malignant features histologically indistinguishable from glioblastoma. 1 The issue of WHO grading of PXA with anaplastic or malignant transformation is unresolved, however many authors use WHO Grade III or IV presumptively. 2 , 3 We describe a case of pleomorphic xanthoastrocytoma with ana-plastic features ina 41-year-old female which demonstrated multiple recurrences over a period of 9 years culminating in transformation to high grade gliomawith sarcomatoidareas.These areasdemonstrated interlacing fasciclesofatypical spindled cells, high mitotic index and focal necrosis and showed positive immunohistochemical staining for smooth muscle actin and pan-actin with relative loss of GFAP expression. The development of sarcomatoid features heralded the onset of more aggressive behaviour, with local recurrence, devel-opmentofmultiple spinal metastases and demise12months later. To our knowledge this pattern of malignant transformation within PXA has only been described once previously. 4

Symptomatic intraventricular xanthogranulomas

Abstract This article reviews symptomatic intraventricular xanthogranulomas, based on a case presentation. Bilateral xanthogranulomas of the choroid plexus were removed surgically from the lateral ventricles of a 12-year-old boy. At 9 years of age, he had evidence of increased intracranial pressure and was hospitalized. Dense enhancing masses were detected in computerized tomogram (CT) brain scan. The lesions were in the region of trigones with extension into the temporal horns and into the right occipital horn. The masses were brightly yellow and greasy. They measured 8.5 x 5.5 x 3.5 cm and 10 x 6.5 x 4.5 cm, respectively, and proved to be xanthogranulomas. Review of 35 reported symptomatic intraventricular xanthogranulomas revealed 11 lesions in the lateral ventricles in which six of them were bilateral. Twenty-two lesions were in the third ventricle, and two lesions were in the fourth ventricle. The lesion shows no significant sexual predilection. The patients’ average age is 37.6 years for males, 32.4 years for females, and 34.3 years for both sexes. The size of symptomatic lesions ranged from 1 to 3 cm in diameter but a few were large, up to 8 to 10 cm. The origin of foamy (xanthoma) cells in the xanthogranulomas arising in the choroid plexus is thought to be multicentric including the choroidal epithelium and stromal arachnoidal cells that have undergone xanthomatous changes. Increased intracranial pressure is the significant clinical feature of the intraventricular xanthogranulomas as in other mass lesions within the skull. Surgical extirpation is the treatment of choice if the lesion is accessible and the patient’s general condition is suitable.

Central Nervous System Disease in Langerhans Cell Histiocytosis

Diabetes insipidus and anterior pituitary dysfunction, are familiar central nervous system (CNS) complications of Langerhans cell histiocytosis (LCH) but the pathophysiology and biological behaviour of other forms of CNS involvement in LCH are poorly understood. In an attempt to improve our understanding of these rare complications, we studied 23 patients with LCH in whom neuroradiological abnormalities, with or without neurological dysfunction other than diabetes insipidus, developed during the course of disease. Neuroradiological abnormalities were of three basic types (a) poorly-defined changes in white matter, (b) well-defined changes in white and grey matter and (c) extra-parenchymal "tumoural" masses. There was a profusion of associated neurological signs and symptoms in most cases but some patients were asymptomatic. The neuropathological features were complex but infiltration of the CNS by histiocytes with xanthomatous change, particularly prominent in mass lesions, was common in the 13 cases in which biopsies were done. Patients with lytic lesions of the skull and diabetes insipidus are evidently most at risk of developing these rare manifestations of LCH. Therapeutic questions could not be answered from this study because no standard treatment had been given and outcome varied widely.

Carcinoma pseudohiperplásico con cambios xantomatosos: una neoplasia que semeja hiperplasia glandular de la próstata

Introduction and objectives Varieties of prostatic adenocarcinoma whose architectural and cytological appearance mimicked benign lesions have been reported in recent decades. Such neoplasms include xanthomatous (foamy) carcinoma and pseudohyperplastic carcinoma. We recently studied five carcinomas showing a cytoarchitectural combination of both neoplasms which were confused with benign glandular proliferations. Methods Five cases (1.8%) of pseudohyperplastic carcinoma showing xanthomatous changes were selected from a total of 280 biopsies showing prostate carcinoma. Glandular prostatic hyperplasia was originally diagnosed in four of such cases. Results Patient age ranged from 54 and 78 years (mean, 64 years). All patients had high prostate-specific antigen levels, and digital rectal examination showed abnormalities in four of them. Neoplasms showed minimal atypia and consisted of mid- to large-sized glands arranged in nests resembling hyperplastic nodules. Glands showed papillary projections, infoldings, and undulations. Most nuclei were basal, small and hyperchromatic, and nucleomegaly was only seen in two biopsies in isolated histological fields. Several useful criteria for diagnosis of acinar carcinoma, such as perineural infiltration, mitosis, crystalloids, blue secretions, and prostatic intraepithelial neoplasm, were absent. Conclusions Prostatic carcinoma with a pseudohyperplastic pattern and xanthomatous changes mimics hyperplastic glands. Timely detection is critical to avoid treatment delay.

Pseudohyperplastic carcinoma with xanthomatous changes: A neoplasm mimicking glandular hyperplasia of the prostate

Introduction and objectivesVarieties of prostatic adenocarcinoma whose architectural and cytological appearance mimicked benign lesions have been reported in recent decades. Such neoplasms include xanthomatous (foamy) carcinoma and pseudohyperplastic carcinoma. We recently studied five carcinomas showing a cytoarchitectural combination of both neoplasms which were mistaken for benign glandular proliferations. MethodsFive cases (1.8%) of pseudohyperplastic carcinoma showing xanthomatous changes were selected from a total of 280 biopsies showing prostate carcinoma. Glandular prostatic hyperplasia was originally diagnosed in four such cases. ResultsPatient age ranged from 54 to 78 years (mean: 64 years). All patients had high prostate-specific antigen levels, and digital rectal examination showed abnormalities in four. Neoplasms showed minimal atypia and consisted of mid- to large-sized glands arranged in nests resembling hyperplastic nodules. Glands showed papillary projections, infoldings, and undulations. Most nuclei were basal, small and hyperchromatic, and nucleomegaly was observed only occasionally. Several useful criteria for the diagnosis of acinar carcinoma, such as perineural infiltration, mitosis, crystalloids, blue secretions, and prostatic intraepithelial neoplasia, were absent. ConclusionsProstatic carcinoma with a pseudohyperplastic pattern and xanthomatous changes mimics hyperplastic glands. Timely detection is critical to avoid treatment delay.

A Giant Guteal Schwannoma with extension into the Pelvis: A case report

Schwannomas are uncommon slow growing tumours arising from the nerve sheath or Schwann cell. To report a case and the surgical removal of a giant complex schwannoma and to highlight the value of extensive investigations including a preoperative histologic diagnosis in the successful surgical management of uncommon large benign tumours. A 39-year-old man presented with a 15-year history of a large mass in the right gluteal region. He was clinically evaluated, subjected to imaging studies and surgery. Clinical examination revealed a 40 cm by 60 cm mass in the right gluteal region and continuous with a 25 cm by 15 cm pelvic mass. The ultrasound scan revealed a very complex (mixed solid and fluid containing) gluteal mass with extension to the pelvis. The CT scan showed a very large, well defined lobulated tumour with cystic spaces and enhancing nodules. The tumour extended through the ischiadic foramen into the pelvis and posteriorly into the thigh muscles. A Doppler scan of the pelvic vessels revealed that the right common and internal iliac arteries were both compressed but not occluded. An incisional biopsy was reported as a Schwannoma with xanthomatous changes and an immuno histochemistry profile of S-100+, Ki-67+ (less than 1% of the cells). A right foot drop following the surgery responded to physiotherapy. The duration of total hospital stay was 12 weeks. The staged excision of large and complex schwannomas is safe. It is essential that a preoperative histological diagnosis is made to establish that the tumor is truly benign.

An Unusual Case of Intraparotid Facial Nerve Schwannoma

An Unusual Case of Intraparotid Facial Nerve Schwannoma

Neurenteric cyst with xanthomatous changes in the prepontine area: unusual radiological findings

Preoperative diagnosis of neurenteric cysts can be difficult because the imaging findings of a neurenteric cyst may be similar to those of an arachnoid cyst. The authors report a case of a neurenteric cyst with xanthomatous changes in the prepontine area. This 4-year-old girl was admitted to their institution with intermittent neck pain and vomiting. Computed tomography showed a hypodense mass in the prepontine area. Magnetic resonance imaging showed a cystic lesion measuring approximately 4 x 3 cm. The brainstem was displaced posteriorly, and the cisterns in both cerebellopontine angles were widened. The signal intensity of the cyst was similar to that of cerebrospinal fluid. Adjacent to the basilar artery there was a solid component of the mass that enhanced after administration of Gd. Intraoperatively, the authors found a cystic mass containing clear fluid with a yellowish solid nodule. On the basis of histopathological findings, the lesion was diagnosed as a neurenteric cyst with xanthomatous changes.