Giant Cell Research Articles

GIANT CELL MYOCARDITIS: AUTOPSY CONFIRMATION OF A DIAGNOSTIC DILEMMA - A RARE CASE REPORT

Abstract Introduction/Objective Giant cell myocarditis (GCM) is a rare and rapidly progressive form of myocarditis, commonly leading to fulminant heart failure (HF), refractory ventricular arrhythmias or conduction system abnormalities and carrying a high mortality rate without prompt treatment. Due to its rarity, there is limited data on its prevalence, although studies show an incidence of 0.007–0.053% among autopsied cases. This entity primarily affects the heart, exhibiting a rapid clinical course and a poor prognosis necessitating immunosuppressive therapy and, in most instances, heart transplantation to improve survival rates. Methods/Case Report We present a case of a 62-year-old male with a prior history of complete heart block status post permanent pacemaker placement, presenting with acute decompensated heart failure. Laboratory investigations revealed elevated cardiac troponin levels (>35000) with echocardiography showing a reduced ejection fraction of 25% and an initial diagnosis of non-ST elevation myocardial infarction (NSTEMI) was made. Endomyocardial biopsy revealed GCM. Despite aggressive management, including immunosuppressive therapy and mechanical circulatory support, his condition deteriorated rapidly, leading to multiorgan failure and eventual death. The diagnosis was confirmed on autopsy. The diagnostic challenge of GCM lies in its clinical or pathologic overlap with other myocardial disorders such as cardiac sarcoidosis, viral myocarditis, and cardiac amyloidosis. Endomyocardial biopsy (EMB) remains the gold standard for diagnosing this entity which is characterized by a widespread inflammatory cell infiltrate with multinucleated giant cells in association with myocyte damage. The etiology of GCM remains unclear, but it is believed to be immune-mediated, possibly triggered by an autoimmune response. Results (if a Case Study enter NA) NA Conclusion Pathologists should give importance to consider GCM in the differential diagnosis of patients presenting with acute heart failure refractory to standard therapy. Early recognition and prompt initiation of immunosuppressive therapy are crucial for improving outcomes in patients with GCM. Awareness of this entity is crucial to make a timely diagnosis.

Role of the type 6 secretion system on apoptosis and macrophage polarization during Burkholderia pseudomallei infection.

Burkholderia pseudomallei (Bpm) is the causative agent of the disease melioidosis. As a facultative intracellular pathogen, Bpm has a complex lifestyle that culminates in cell-to-cell fusion and multinucleated giant cells (MNGCs) formation. The virulence factor responsible for MNGC formation is the type 6 secretion system (T6SS), a contractile nanomachine. MNGC formation is a cell-to-cell spread strategy that allows the bacteria to avoid the extracellular immune system and our previous data highlighted cell death, apoptosis, and inflammation as pathways significantly impacted by T6SS activity. Thusly, we investigated how the T6SS influences these phenotypes within the macrophage and pulmonary models of infection. Here we report that the T6SS is responsible for exacerbating apoptotic cell death during infection in both macrophages and the lungs of infected mice. We also demonstrate that although the T6SS does not influence differential macrophage polarization, the M2 polarization observed is potentially beneficial for Bpm pathogenesis and replication. Finally, we show that the T6SS contributes to the severity of inflammatory nodule formation in the lungs, which might be potentially connected to the amount of apoptosis that is triggered by the bacteria.

PERITONEAL KERATIN GRANULOMAS MIMICKING PERITONEAL CARCINOMATOSIS: A POTENTIAL DIAGNOSTIC PITFALL

Abstract Introduction/Objective Keratin granulomas from a primary uterine cancer can present as peritoneal tumor nodules, mimicking peritoneal carcinomatosis. Endometrial adenocarcinomas with viable peritoneal tumor implants and/or involvement of bladder or bowel mucosa are categorized as stage IV disease. We present a rare case of endometrial adenocarcinoma of the uterus with squamous differentiation and multiple peritoneal nodules of keratin granulomas, masquerading as peritoneal carcinomatosis. Methods/Case Report A 68-year-old female with insignificant past medical history presented with complaints of postmenopausal bleeding for the past four months. Physical examination was unremarkable. Transvaginal ultrasound showed a 4.9 cm echogenic mass in the uterus. The endometrial biopsy showed squamous debris with atypical cells suspicious of malignancy. Endometrial curettings revealed endometrial adenocarcinoma, endometrioid type with squamous differentiation, FIGO Grade 1. Subsequently, she underwent hysterectomy with bilateral salpingo- oophorectomy. Intraoperatively, multiple suspicious nodules on the sigmoid colon and in the pelvis were identified, of which excisional biopsies and pelvic peritoneal washing were taken. Microscopic examination of the resection confirmed endometrial adenocarcinoma, FIGO Grade 1, nuclear grade 1, with extensive foreign body giant cell reaction to dead squamous cells (ghost cells). Cytologic examination of the peritoneal washing was unremarkable and excisions of the sigmoid serosal and peritoneal nodules showed pink eosinophilic laminated nodules surrounded by inflammatory cells including plasma cells, lymphocytes and multinucleated giant cells without any viable tumor cells. Results (if a Case Study enter NA) NA Conclusion Keratin granulomas are a foreign body reaction to keratin deposition from the squamous component of endometrioid adenocarcinoma. The pathogenesis of this foreign body response to keratin is considered to be a trans- tubal spread of keratin through retrograde shedding from the fallopian tubes. In the absence of viable tumor cells, it appears to have no negative prognostic significance. Careful microscopic examination of peritoneal keratin granulomas is imperative to avoid erroneous upstaging of the tumor and the subsequent advanced treatment.

Dual infection of maxillary sinus masquerading as a malignant lesion; A rare presentation

Primary sinonasal tuberculosis is a rare form of TB even in developing countries where TB is prevalent and invasive mycotic infection is more common in immunocompromised patients. It is unusual to have a dual infection with tuberculosis and mycosis in the maxillary region mimicking a neoplastic process and causing bone erosion. A 49-year old male presented with 2-year history of left-sided facial swelling. On microscopic examination, epithelioid cell granulomas, chronic inflammatory infiltrate, macrophages, giant cells, and focal necrosis were seen. Ziehl Neelsen staining for acid-fast bacilli with 20% H2SO4 was positive. Fungal hyphae were also seen in the sections examined. Special stains (Periodic acid-Schiff and Grocott’s methenamine silver stain) for fungal hyphae were positive. Mycosis and tuberculosis should always be considered in the differential diagnosis of maxillary lesions or sinusitis.

An Anaplastic Lymphoma Kinase (ALK) Immunohistochemical Pitfall: ALK-positive Histiocytosis versus Angiomatoid Fibrous Histiocytoma

Abstract Introduction/Objective The 5th Edition WHO classification expanded the histiocytic/dendritic cell neoplasms to include ALK-positive histiocytosis, which can present as multisystem or single-site disease, affecting various age groups. These tumors lack high-grade cytologic features but express ALK by immunohistochemistry, typically indicating ALK locus rearrangements. In this case study, a cutaneous mass initially suspected as ALK-positive histiocytosis was found to be angiomatoid fibrous histiocytoma (AFH) with ALK expression, highlighting the potential diagnostic pitfall due to overlapping histomorphology and ALK expression. Methods/Case Report A previously healthy twelve-year-old boy sought evaluation for a growing and ulcerating mass on his left arm, which developed gradually over a year post-injury. Laboratory tests showed elevated inflammatory markers and mild anemia. A biopsy of the ulcer revealed Staphylococcus aureus infection. Initial histological examination revealed mixed inflammatory infiltrates with focal necrosis, prominent histiocytes, and giant cells. Despite favoring an infectious cause, immunostains were performed. AE1/AE3, ERG, CD34, CD30, S100, SOX10, SMA, and MyoD1 were negative, while INI1 was retained. However, ALK was strongly positive in some histiocytoid cells, suggesting ALK-positive histiocytosis. Surgical resection was subsequently performed to manage the abscess and characterize the lesion. Histologic examination revealed a solid nodule containing spindled and epithelioid cells with a bland histiocytoid appearance, arranged in a compact syncytial growth pattern within the dermis and subcutis. Surrounding the tumor, there was a distinct cuffing of fibrosis with organized lymphoplasmacytic cells. Break apart fluorescence in situ hybridization studies showed rearrangement of EWSR1 but not ALK. The diagnosis of angiomatoid fibrous histiocytoma was rendered. Results (if a Case Study enter NA) NA Conclusion This case highlights the importance of understanding immunohistochemical features, especially those that are unusual and unexpected in rare diseases. With the growing use of minimally invasive sampling techniques, this case study underscores the significance of integrating molecular diagnostics with clinical, histological, and immunophenotypic evaluations to achieve an accurate diagnosis in surgical pathology.

Urogenital schistosomiasis, a leading cause of bladder squamous cell carcinoma

Abstract Introduction/Objective Schistosoma, genus of trematodes, commonly known as blood flukes are parasitic flatworms responsible for a group of infections in humans termed schistosomiasis and hundreds of millions of people are infected worldwide. In areas where Shistosoma is not endemic to the region most cases of bladder malignancy are of urothelial origin. In areas where Shistosoma is endemic bladder malignancy is more commonly squamous cell carcinoma. However, with increased travel, migration and tourism it is important to potentially consider Shistosoma infection in areas that are not typically considered endemic regions. Methods/Case Report A 62-year-old male presented to our hospital and revealed through an interpreter he has had 1.5 years hematuria with “pieces of meat or brown dirt”. Cystoscopy was performed and showed a large necrotic mass. CT scan showed an 8.2 cm exophytic bladder dome mass. TURBT was performed and a diagnosis of invasive high-grade urothelial carcinoma with squamous differentiation and with multiple Schistosoma organisms identified.The patient received Schistosoma treatment and was scheduled for radical cystectomy and ileal conduct. A few days before the scheduled procedure the patient presented to our emergency department with acute abdominal pain. He was subsequently diagnosed with progressive bladder cancer with new left malignant ureteral obstruction, which lead to hydronephrosis, DVT, abdominopelvic ascites and intraperitoneal bladder perforation. Abdominal fluid cytology showed atypical keratinizing squamous cells suspicous for malignancy and marked acute inflammation. Patient underwent cystectomy with ileal conduit and the specimen was sent to pathology for examination. Results (if a Case Study enter NA) NA Conclusion Gross examination showed an irregular perforated bladder. Microscopic examination showed a high grade urothelial carcinoma with squamous and sarcomatoid differentiation with detrusor muscle invasion. Additionally, Schistosoma organisms with a terminal spine and calcified eggs were identified. Some of the calcified egg material was engulfed by giant cells. The organisms are surrounded by a prominent eosinophilic infiltrate. Lymphovascular invasion is also noted. Schistosomiasis is associated with the development of squamous cell carcinoma of the bladder. It is important to maintain high oncological alertness for squamous cell carcinoma, particularly for patients arriving from endemic areas. Such as in our case, a patient from Somalia living in Kansas City.

ALK-Positive Histiocytosis and its Distinctive Manifestation in the Breast

Abstract Introduction/Objective ALK-positive histiocytosis (APH) is a rare histiocytic proliferation disease that harbors ALK rearrangements, often involving fusion with KIF5B. Primary cases in the unilateral breast are exceptionally uncommon, with only a few reported cases globally, characterizing it as a unique histiocytic proliferation disorder. Methods/Case Report In our study, we report a case of unilateral multifocal breast involvement of ALK-positive histiocytosis following bilateral breast core biopsies in a 56-year-old Hispanic woman who presented with bilateral breast masses. Right breast imaging demonstrated multiple nodular densities, further characterized on ultrasound as a hypoechoic region of 8 cm with increased vascularity. On right breast biopsy, prominent proliferation of histiocytes (lacking high-grade cytologic atypia) with chronic inflammation, few giant cells, and focal cholesterol clefts were identified. On immunohistochemical studies, the lesional cells were variably positive for histiocytic markers CD68, CD163, and PU.1 and were negative for S100 and pancytokeratin. ALK1 showed multifocal staining in the lesional cells as confirmed on repeat study; however, ALK (D5F3), GMS and acid fast studies were negative. The presence of focal cholesterol clefts, scattered giant cells, and chronic inflammation lead us to consider aberrant ALK expression in a reactive process. However, APH was definitively diagnosed through confirmation of clonal histiocyte proliferation, observed findings on morphology, immunophenotyping, and the presence of KIF5B-ALK gene fusion as detected by comprehensive solid tumor fusion panel testing. Results (if a Case Study enter NA) NA Conclusion It is noteworthy that ALK-positive histiocytosis can occasionally present alongside features of a reactive process in the breast. Therefore, considering any history of prior surgery or trauma at the lesion site is crucial. However, we recommend a comprehensive approach in most cases, which includes morphological, immunohistochemical, and molecular analysis on all suspected histiocytosis cases. This approach aims to increase awareness and detection of this rarely described entity and to precisely distinguish it from other diseases for prognostic and therapeutic purposes.

Pulse Granuloma in Esophagus; A Rare finding in Achalasia Patient: Case Report and Literature Review

Abstract Introduction/Objective Pulse granuloma is a granulomatous reaction to foreign body, most commonly to food material. This is clinically silent and incidental but may be confused for a mass like lesion. This can be found any location in gastrointestinal tract but most reported in oral cavity and colon. We herein report a rare case of pulse granuloma in esophagus with literature review of the entity. Methods/Case Report A 58-year-old male with history of achalasia, surgical history of Heller myotomy, hernia reduction, hiatoplasty, gastropexy, vagotomy, presented with recurrent stricture of the esophagogastric junction. The patient received multiple esophageal balloon dilatations in the past due to high esophageal sphincter pressure and was complicated by severe reflux associated with esophagitis and large hiatal hernia. He underwent subtotal esophagectomy due to dysphagia. Gross examination of the esophagectomy specimen showed multiple previous procedure scars and was opened to reveal firm yellow food particles impacting in the mucosa. Histological examination showed gastroesophageal junctional mucosa with ulceration and fibrosis of the muscularis propria. Pink ribbon like hyalinized foci with giant cell was found which stained negative for GMS, PAS and AFB, supporting the diagnosis of pulse granuloma. Literature search for key word ‘Pulse Granuloma’ identified thirty-five case reports from year 1977 to 2024, with most reported case in the oral cavity (14 cases, 42%). Out of ten cases reported in gastrointestinal tract, 1 case was reported in small bowel and 9 cases were reported in colon and rectum and no cases were reported in esophagus. Results (if a Case Study enter NA) NA Conclusion Our patient’s history of achalasia and dysphagia along with histopathologic findings supports diagnosis of pulse granuloma in esophagus. Although clinically silent, pulse granuloma may cause mass-like lesions and result in enlargement and stenosis. In setting of esophageal achalasia recognizing this entity is important as it can mimic malignancy and other mass-forming lesions.

An idiosyncratic reaction to the synthetic biologic membrane on a surgical wound

Abstract Introduction/Objective Biodegradable temporizing matrix (BTM) is a synthetic polymer used to temporarily close wounds and aid the body in generating new tissue. BTM is applied to a debrided wound.BTM acts as a scaffold aiding the migration of granulation tissue into the matrix. Once the tissue is fully integrated throughout the BTM, the sealing membrane is removed. The matrix slowly deteriorates until fully absorbed at approximately 18 weeks. Methods/Case Report An 80-year-old woman with a past medical history of eczema, rheumatoid arthritis and gout presented with swelling of the left third metatarsal. Imaging showed an abscess which was eventually drained and cultured to show mycobacterium chelone. The patient was started on antibiotics for mycobacterium. On follow up she reported a nodule on left forearm recently increased in size. MRI on 9/28 showed abscess of the dorsal aspect of phalanx. She underwent surgical debridement 10/31 on her finger and the forearm nodule. After debridement a polynovo BTM graft was placed. Histopathology showed skin and subcutaneous tissue with necrotizing granulomas of the finger and the forearm. Cultures and stains for mycobacterium returned negative.The patient was subsequently followed up for wound healing. She reported nodules on the left forearm at the scar from previous surgical site which were biopsied. Histopathology showed granulomatous inflammation, rare foreign body giant cells, and birefringent material. The stains for Ziehl-Neelsen, Fite and Gomori’s Methenamine Silver for Urate Crystals were all negative excluding the possibility of gouty tophi or mycobacterium. Results (if a Case Study enter NA) NA Conclusion The complex history of the patient with mycobacterium, gout, the giant cell reaction and the unique appearing birefringent crystals presented a puzzling problem. Given the negative stain results for mycobacteria and the presence of birefringent (foreign) material, we deem these granulomas most likely an idiosyncratic reaction to the synthetic biologic membrane applied during her prior procedure. There are no known cases of giant cell reaction and similar morphology of birefringent (foreign) material in literature.

Inflammation of the temporalis muscle and adjacent nerve tissue in giant cell arteritis: expanding the spectrum of inflammatory lesions.

To investigate the histopathological features of the temporalis muscle (TM) and adjacent nerve tissue in active cranial giant cell arteritis (C-GCA). Temporal artery biopsy (TAB) specimens containing fragments of the TM from patients with active C-GCA fulfilling the 2022 ACR/EULAR classification criteria (n = 11) were assessed by conventional histology and immunohistochemistry in comparison with non-GCA controls (n = 3). Clinical, laboratory and imaging features based on patient charts at time of biopsy were retrospectively recorded. The majority of the studied TAB specimens showed inflammation of the TM (10/11) and adjacent nerve fascicles (7/11) that was characterized by prominent endomysial lymphomonocytic infiltrates, whereas controls showed no inflammatory lesions and no disruption of the local architecture. Association of active C-GCA with sarcolemmal MHC class I (8/8) and MHC class II (6/11) upregulation suggests primary inflammation of the TM in a subset of patients. αB-Crystallin positivity (10/11) highlights areas of pre-necrotic myofibres within the TM. The presence of endomysial fibrosis, signs of atrophy and variations of muscle fibre size suggest a rather longstanding and potentially subclinical process of myoinflammation. Our results expand the spectrum of inflammatory lesions known to be associated with active C-GCA. Specifically, inflammatory infiltration of the TM and adjacent nerve structures could contribute to localized symptoms of the temporomandibular region and may be included in future concepts of pathophysiology.

Polymyalgia rheumatica and giant cell arteritis: diagnosis and management.

There have been advances in the diagnosis and treatment of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR). Themes in PMR and GCA include classification criteria, ultrasound imaging of temporal and axillary arteries replacing biopsies for diagnosis of GCA, faster diagnosis and treatment with rapid access clinics for suspected GCA, and expanding treatment options with the goal of rapid suppression of inflammation and sparing steroids. Treatment is aimed at suppressing inflammation quickly in both GCA and PMR. Randomized trials have demonstrated success in reducing glucocorticoids when adding advanced therapies such as interleukin 6 (IL6) inhibitors. Other treatments including Janus kinase (JAK) inhibitors (especially a phase 3 trial of upadacitinib at 15 mg daily and secukinumab (an IL17 inhibitor) are being tested. Some uncontrolled GCA protocols are limiting glucocorticoids to initial IV pulse therapy only or rapid tapering of oral glucocorticoids with upfront treatment with tocilizumab. There is uncertainty of who should have an advanced therapy and how long to use it for and what order to consider advanced therapies when treatment fails. In PMR, studies are performed when patients cannot taper glucocorticoids effectively, whereas in GCA, advanced therapies are started with disease onset or with recurrent GCA.

Rectal Schistosomiasis and Adenocarcinoma

Abstract Introduction/Objective Schistosoma infestation has been implicated by the International Agency for Research on Cancer as an etiology for several cancers such as colon, liver, and bladder. S. japonicum is classified as a probable carcinogen in humans (class 2B). These trematodes may cause chronic infection in the affected organ leading to the trend of “inflammation-adenoma-carcinoma”. There is rising evidence that infection with Schistosoma japonicum is correlated with the liver and colorectal carcinoma in endemic Asian countries. We herein present a case of rectal adenocarcinoma associated with Schistosoma as a possible causal factor. Methods/Case Report An 88-year-old-woman from China, currently living in United States for past six years, presented with rectal bleeding. Colonoscopy showed evidence of a rectosigmoid mass 2.2 x 1.0 x 0.3 cm. She underwent trans-anal excision of the tumor. Microscopic examination showed moderately differentiated adenocarcinoma with tumor invading the muscularis propria (pT2). There were numerous oval / round calcified eggs (30 - 46 microns) with minute subterminal or no spine morphologically consistent with Schistosoma japonicum associated with granulomatous and foreign body giant cell reaction, fibrosis, and tissue eosinophilia. She was treated with Praziquantel and is currently undergoing endoscopic surveillance for recurrence. Results (if a Case Study enter NA) N/A Conclusion Patients with chronic Schistosoma japonicum infection have 3 times increased risk of developing colorectal cancer than those with no previous exposure to schistosomal infection. We believe that chronic S. japonicum infection is the likely etiology of rectal cancer in our patient. Physicians should be aware of such associations, especially for immigrants from endemic areas, to facilitate accurate diagnosis and management.

SWI/SNF Altered Gastric Carcinoma with Squamous Differentiation in a young patient with aggressive clinical course

Abstract Introduction/Objective Inactivation of SMARCA4 (BRG1) protein subunit of the SWI/SNF complex has been reported in various malignancies affecting multiple organs, often associated with a poor prognosis. We report a rare case of gastric SMARCA4-deficient carcinoma in a young patient, showing squamous differentiation and an aggressive clinical course. Methods/Case Report A 28-year-old male presented with epigastric pain and 15-lb weight loss over 3 months. Evaluation revealed a gastric cardia mass, liver lesion, and gastro-hepatic lymph-node. Biopsy of gastric and liver lesions revealed a poorly differentiated carcinoma. Following a course of neoadjuvant chemotherapy, and immunotherapy, total gastrectomy and distal esophagectomy were performed. Grossly, there was a 13 cm mass involving his gastric cardia and body, extending into his distal esophagus. On microscopy, the transmural tumor was arranged as solid sheets, syncytial aggregates, and nests with a focal pseudoglandular pattern in a lymphoplasmacytic rich stroma. Scattered bizarre giant cells were noted, along with brisk mitosis and focal necrosis. Focally, the tumor showed abundant glassy eosinophilic cytoplasm reminiscent of squamoid cells. Two of forty-two lymph nodes were positive for metastatic carcinoma. IHC showed focal positivity for AE1/3, OSCAR, CAM5.2, along with areas staining for p40 and p63; negative for CK7, CK20, CDX2, SATB2, synaptophysin, INSM1, SOX-10, LCA ERG, and SALL4. Nuclear expression for mismatch repair proteins was retained. HER2 was negative (score 0), and PDL-1 CPS score was 40. EBER in-situ hybridization was negative, with retention of INI-1. There was complete loss of SMARCA4 by IHC and next generation sequencing confirmed frameshift alteration of SMARCA4. Final pathologic diagnosis was SMARCA4 deficient poorly differentiated carcinoma with squamous differentiation (ypT3pN1). The patient received adjuvant therapy with Paclitaxel and Ramucirumab. However, his disease progressed, and he succumbed to his illness five months after surgery. Results (if a Case Study enter NA) N/A Conclusion Our case illustrates that SMARCA4 deficient gastric carcinomas can have heterogeneous morphology, with an aggressive clinical course in young patients. This highlights the importance of early diagnosis and consideration of alternative therapeutic approaches, as these tumors typically do not respond to standard treatments.

Multisystem ALK-Positive Histiocytosis With DCTN1::ALKFusion in an Adult, Responsive to Alectinib: Case Report and Literature Review.

Anaplastic lymphoma kinase (ALK)-positive histiocytosis has emerged as a clinically relevant diagnosis featuring a wide span of clinical presentations, which are unified by the presence of ALK-positive histiocytes on histopathology and molecular drivers involving the ALK kinase gene. This report presents an adult case of multisystem ALK-positive histiocytosis with xanthogranuloma-like features on histopathology that was responsive to ALK inhibition, and includes a review of ALK-positive histiocytoses with cutaneous involvement reported in the literature. A 56-year-old male developed a widespread eruption of red-brown papules on the face, trunk, and upper extremities. Histopathological evaluation revealed a well-circumscribed, nodular dermal infiltrate of epithelioid histiocytes with Touton giant cells, rare bizarre multinucleated cells, and focal emperipolesis. The lesional cells were positive for CD68 and ALK1 immunohistochemical stains, and negative for CD1a. Next-generation sequencing identified a DCTN1::ALK fusion. On imaging, he was found to have bone, lung, soft tissue, and salivary gland involvement. ALK inhibition was initiated with alectinib, resulting in rapid improvement of cutaneous lesions and eventual complete resolution of abnormal imaging findings, which was sustained at 24 months of follow-up. This case adds to the spectrum of ALK-positive histiocytoses and further demonstrates the positive response with targeted therapy.

Surgical Management of 2,350 Pediatric Dermoid Cysts.

We examined operative and pathologic findings of a large series of dermoid cysts at a high-volume pediatric hospital over 23 years. A retrospective review was performed of all dermoid cysts excised from 2000 to 2023 at the Children's Hospital of Philadelphia. Lesions were classified by location. Depth was stratified into Type 1, superficial; Type 2, subperiosteal or containing a stalk to a cranial suture; Type 3, intraosseous or intracartilaginous; Type 4, intracranial extradural; and Type 5, intracranial intradural. Of 2,350 lesions, 2,237 (95.2%) were in the head and neck. Most common locations were lateral brow and orbit, 892 (38%); anterior neck, 303 (12.9%); and frontal, 253 (10.8%). Among the series, 67.9% were Type 1, 10.1% were Type 2, 16.5% were Type 3, 2.3% were Type 4, and 3.2% were Type 5. Older age at surgery correlated with depth among locations demonstrating intracranial extension (r=.061, p=.016). Anterior fontanelle (59.1%), nasal (16.2%), occipital (5.6%), and temporal (4.7%) lesions had the highest intracranial extension rates. Temporal (49.4%), frontal (32.8%), nasal (29.9%), and occipital (22.2%) lesions had higher rates of osseous/cartilaginous involvement. On histopathologic examination, 403 (17.1%) were ruptured. Ruptured lesions were associated with giant cell reaction (46.4 versus 5.7%, p<.001). Anterior fontanelle, nasal, occipital, and temporal lesions are at higher risk of intracranial extension and may require preoperative imaging. Frontal and parietal lesions have a lower risk of intracranial involvement. Lateral brow and orbit, periauricular, and anterior neck lesions demonstrate a higher rate of osseous involvement without intracranial tracking.

Serial assessment of ultrasound sensitivity and scores in patients with giant cell arteritis before and 3 and 10 days after treatment.

To evaluate sensitivity and scores of vascular ultrasound (US) before and after initiating glucocorticoid (GC) treatment in patients with new-onset giant cell arteritis (GCA). Treatment-naïve patients with GCA were prospectively included. 18F-FDG PET/CT, US and temporal artery (TA) biopsy were performed in all patients. US was repeated 3 and 10 days after GC commencement. Intima-media thickness and presence of halo signs were assessed. Sonographers were unblinded to clinical data. The OMERACT GCA Ultrasonography score (OGUS) and halo count (HC) were calculated. Forty-eight patients were included. Before GC exposure, US sensitivity was 94% (95% CI: 83-99), 73% (95% CI: 58-85) and 71% (95% CI: 56-83) when assessing all vessels, TAs, and large vessels (LVs), respectively. At day 3 and 10, overall US sensitivity was 92% (95% CI: 78-98, p= 0.16) and 83% (95% CI: 69-92, p= 0.10), respectively. At day 10, TA-US and LV-US sensitivity was 53% (95% CI: 38-68, p< 0.01), and 60% (95% CI: 44-74, p= 0.13), respectively. Median OGUS decreased from 1.06 (IQR 0.83-1.24)-0.95 (IQR 0.78-1.14, p< 0.01), and 0.90 (IQR 0.73-1.01, p< 0.001) after 3 and 10 days, respectively. Median HC decreased from 3 (IQR 2-5)-2 (IQR 1-4, p< 0.01) after 10 days. The vasculitic US findings expressed by OGUS diminish after 3 days of GC treatment. TA-US sensitivity decreased after 10 days, whereas LV-US was less likely to change, highlighting the importance of LV-assessment. Consistent with the EULAR recommendations, these findings encourage prompt US assessment, preferably within 3 days, to ensure an accurate diagnosis.

An overview of 18F-fluorodeoxyglucose positron emission tomography/computed tomography in giant cell arteritis.

PET/CT is an imaging modality that is increasingly being used to diagnose large-vessel vasculitis. In the case of giant cell arteritis, it was first used to demonstrate inflammation of the walls of large arterial trunks such as the aorta and its main branches, showing that aortic involvement is common in this vasculitis and associated with the occurrence of aortic complications such as aneurysms. More recently, with the advent of digital PET/CT, study of the cranial arteries (i.e., temporal, occipital, maxillary and vertebral arteries) has become possible, further increasing the diagnostic interest of this examination for the diagnosis of GCA. Despite these advantages, there are still limitations and questions regarding the use of PET/CT for the diagnosis and especially the follow-up of GCA. The aim of this review is to take stock of currently available data on the use of PET/CT for GCA diagnosis and follow-up.

Surgical Treatment of Primary Spinal Tumors

Primary spinal tumors appear to be much less common than metastatic lesions, but their surgical treatment comprises a complex and multifaceted task. Numerous factors influence indications and timing of surgical intervention, including neurological status of the patient, histological characteristics of the tumor, its localization, stability of the spinal column, and comorbidities. Significant spinal cord compression, rapid progression of neurologic deficits, or pronounced instability of the spinal column may require urgent surgical intervention. When the spinal canal is not affected, treatment should start with a biopsy to accurately determine the histology of the tumor. Some tumor types, such as giant cell tumors, osteoblastomas, chordomas, and chondrosarcomas, require complete removal of the tumor. However, performing a wide resection in a single block is often found difficult due to compression of vascular and nerve structures. Current approaches to surgical management of primary spinal tumors involve minimally invasive techniques that significantly improve postoperative recovery and reduce the risk of complications. These techniques were originally used to treat degenerative spinal diseases and trauma; however, they have also demonstrated their effectiveness in tumor surgery. Adapting surgical strategy based on histology and tumor location, as well as integrating minimally invasive techniques, can improve patient survival and quality of life. The present paper describes the latest advances in the surgical treatment of primary spinal tumors, discusses current techniques and strategies, and prospects for further research in this area.

Deep Fungal Infections of Skin and Role of Histopathology in Diagnosis

Abstract Introduction: Deep mycoses acquired by penetrating trauma to the skin can have varied and sometimes atypical morphological presentations resulting in diagnostic dilemmas and delay in treatment onset. Histopathology can be a useful tool in not only diagnosing but also differentiating various deep mycoses. Aims and Objectives: To observe various morphological presentations and histopathological features of deep fungal infections. Materials and Methods: A retrospective multi-centric study was conducted from 2010 to 2020 at 16 centres. The cases with diagnoses of various deep mycoses were included in the study. The patients presenting with cutaneous manifestations were included in the study. Their demographic details, history, presenting signs and symptoms, morphological presentations, histopathological features and treatment details were collected from the case sheets. Results: A total of 124 cases were found from the case records. The most common type was chromoblastomycosis (42) followed by mycetoma (28) and rhinosporidiosis (17). The mean age was 43.76 ± 5.44 years. The average duration of symptoms before presentation was between 2 months to 10 years (average 2.5 ± 1.33 years). Male to female ratio was 1:0.7. Prior history of trauma was recorded in 36% of cases. Chromoblastomycosis cases presented with verrucous to atrophic plaques with black dots on the surface and histopathology findings included pesudoepitheliomatous hyperplasia, epithelioid cell granulomas, copper penny bodies within granulomas and abscesses. Rhinosporidiosis cases had polypoid grape-like lesions in the nose and eyes most commonly with histopathology findings of abundant thick-walled sporangia in dermis packed with thousands of spores. Eumycetoma patients had pigmented, indurated swelling with multiple sinuses discharging black granules and histopathology showed dermal abscesses and foreign body granulomatous reaction with PAS-positive hyphae. Histoplasmosis patients presented with few to multiple nodulo-pustular lesions on skin and palatal ulcers while small basophilic bodies packed in the cytoplasm of histiocytes were noted in histopathology. Phaeohyphomycosis cases presented as deep-seated cystic lesions and biopsy revealed deepithelialized cysts in the dermis or hypodermis with lumen showing necro inflammatory debris and fungal hyphae. Sporotrichosis cases had erythematous, tender nodules and papules either as single lesions or as multiple lesions arranged in a linear fashion and histopathology showed pseudoepitheliomatous hyperplasia of epidermis, loose to well-defined epithelioid cell granulomas and microabscesses. Spores were found in two cases. Cryptococcosis patient had multiple umbilicated lesions resembling giant molluscum contagiosum loose epithelioid cell granulomas and medium-sized spores lying in both intra and extracellularly on histopathology. Penicilliosis patients had nodulo-pustular lesions and histopathology showed an admixture of histiocytes, epithelioid cells, plasma cells, lymphocytes and polymorphs in the dermis with the presence of yeast-like spores in the cytoplasm of histiocytes and epithelioid cells. Entomophthoromycosis cases presented with asymptomatic subcutaneous firm swelling with loss of skin pinchability. Conclusion: Though clinical findings of deep fungal infections are characteristic similar morphology and atypical presentations can be sometimes confusing. Histopathology is useful for confirming the diagnosis.

Treatment Modalities for Refractory-Recurrent Tenosynovial Giant Cell Tumor (TGCT): An Update

Background and Objectives: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive, benign neoplasm arising from the synovium of joints, tendon sheaths, and bursa. There are two main subtypes of TGCT: localized-type TGCT(L-TGCT) and diffuse-type TGCT (D-TGCT). While surgical excision is still considered the gold standard of treatment, the high recurrence rate, especially for D-TGCT, may suggest the need for other treatment modalities. Materials and Methods: This study reviews current literature on the current treatment modalities for refractory-relapsed TGCT disease. Results: The gold standard of treatment modality in TGCT remains surgical excision of the tumor nevertheless, the elevated recurrence rate and refractory disease, particularly in D-TGCT indicates and underscores the necessity for additional treatment alternatives. Conclusions: TGCT is a benign tumor with inflammatory features and a potential destructive and aggressive course that can lead to significant morbidity and functional impairment with a high impact on quality of life. Surgical resection remains the gold standard current treatment and the optimal surgical approach depends on the location and extent of the tumor. Systemic therapies have been recently used for relapsed mainly cases.