Subcutaneous Leiomyosarcoma: An Aggressive Malignancy Portending a Significant Risk of Metastasis and Death.

Subcutaneous leiomyosarcoma (LMS) is a rare, poorly understood variant. The current literature on the subject is sparse, consisting of isolated case reports and small clinicopathologic studies compromised by the inclusion of both its more common and indolent counterpart, cutaneous LMS (atypical intradermal smooth muscle neoplasm), as well as highly aggressive deep-seated tumors. Thus, precise clinicopathologic characterization is limited. Cases of subcutaneous LMS reviewed at the University of Michigan and Cleveland Clinic from 1994 to 2022 were included in this retrospective study. A total of 39 cases were identified. The mean age was 61 years, and the cohort was predominantly male (23/39; 59%). Tumors averaged 4.2 cm and most commonly arose on the extremities (32/39; 82%). The majority (38/39; 97%) were diagnosed at an early pathologic stage (pT1 or pT2). Histopathologically, most tumors were well-circumscribed and were assigned a Fédération Nationale des Centers de Lutte Contre le Cancer grade of either 1 or 2 (24/39; 62%). The majority (22/39; 56%) appeared to arise in association with a blood vessel. Of the 36 cases with accessible clinical data and follow-up (mean 34 mo, range 0 to 94 mo), 12 (33%) were noted to have metastasized, with the lung representing the most common anatomic location. One case recurred locally. Six of 36 patients (17%) died from the disease at an average of 47 months after diagnosis (range 16 to 94 mo). Metastasis or death from disease was significantly associated with the Fédération Nationale des Centers de Lutte Contre le Cancer grade ( P =0.0015), the presence of necrosis ( P =0.032), tumor size ( P =0.049), and AJCC tumor stage ( P =0.036). These data demonstrate that subcutaneous LMS are more aggressive than dermal-based tumors and have a prognosis akin to that of deep-seated LMS.

Preoperative, Intraoperative, and Postoperative Parathyroid Pathology: Clinical Pathologic Collaboration for Optimal Patient Management.

Parathyroid disease typically presents with parathyroid hyperfunction as result of neoplasia or a consequence of non-neoplastic systemic disease. Given the parathyroid gland is a hormonally active organ with broad physiologic implications and serologically accessible markers for monitoring, the diagnosis of parathyroid disease is predominantly a clinical pathologic correlation. We provide the current pathological correlates of parathyroid disease and discuss preoperative, intraoperative, and postoperative pathology consultative practice for optimal patient care.

Epstein-Barr virus-associated smooth muscle tumors in immunocompromised patients: Six case reports.

BACKGROUND: Epstein-Barr virus associated smooth muscle tumor (EBV-SMT) is a rare oncological entity. However, there is an increasing incidence of EBV-SMTs, as the frequency of organ transplantation and immunosuppression grows. EBV-SMT diagnosis relies on histopathology and immunochemical staining to distinguish it from post-transplant lymphoproliferative disorder (PTLD). There is no clear consensus on the treatment of EBV-SMTs. However, surgical resection, chemotherapy, radiation therapy, and immunosuppression reduction have been explored with varying degrees of success. CASE SUMMARY: Our case series includes six cases of EBV-SMTs across different age groups, with different treatment modalities, adding to the limited existing literature on this rare tumor. The median latency time between immunosuppression and disease diagnosis is four years. EBV-SMTs present with variable degrees of aggressiveness and seem to have worse clinical outcomes in patients with tumor multiplicity and worse immunocompetency. CONCLUSION: It is imperative to continue building on this knowledge and keeping EBV-SMTs on the differential in immunocompromised individuals.

Urticarial vasculitis: A potential signpost for multisystem inflammatory syndrome in children.

Multisystem inflammatory syndrome in children (MIS-C) is a rare and serious complication of Sars-Cov-2 infection. Dermatologic manifestations are present in the majority of patients. Skin lesions found in children with MIS-C are classified into four categories: morbilliform, reticulated, scarlatiniform, and urticarial lesions. Clinicopathologic characterization within these categories is limited. Thus, we present a clear example of an urticarial lesion in the context of MIS-C with well-documented clinicopathologic phenomena. A previously healthy 16-year-old female presented with 3 weeks of an itchy, burning rash initially presenting on her right forearm (and lasting greater than 24 hours without migration) before spreading diffusely. She also reported fever, cough, myalgias, nausea, and vomiting of 4 weeks' duration. Physical examination revealed an edematous, maculopapular, nonblanching, erythematous rash covering the patient's upper extremities, abdomen, back, anterior thighs, and face. The patient tested positive for COVID-19. A low-grade leukocytoclastic vasculitis was noted along with intraluminal fibrin and rare microthrombi in vessels of the mid to deep dermis. The patient was diagnosed with MIS-C and urticarial vasculitis. She was treated with steroids and naproxen for subsequent MIS-C flares. Dapsone treatment was started for the urticarial vasculitis.

Fortuitous Eradication of an Aggressive Basal Cell Carcinoma Via Foreign Body Reaction to a Polyurethane Vacuum-Assisted Closure Sponge.

ABSTRACT: The foreign body reaction (FBR) is a well-documented immune reaction. Much of the literature on FBRs has focused on minimizing this immune response to mitigate the impact on medical implants. Here, we present a case that illustrates a serendipitous oncologic outcome from an FBR. A 54-year-old man presented with an aggressive basal cell carcinoma (BCC). At the first resection, he had broadly positive surgical margins. The surgical wound was temporized with a polyurethane wound vacuum assisted closure (VAC) device. He was lost to follow-up having retained a VAC sponge for a total of 12 weeks. A wide re-resection was performed 7 months after the initial resection. Exhaustive examination of the resected specimen was performed. There was an absence of any BCC, replaced by a widespread chronic FBR to polyurethane VAC sponge particles. This suggests that the foreign body immune response was sufficiently intense to eradicate any remaining BCC. This case illustrates the concept of an FBR as a novel method of local immunotherapy.

Hidradenitis suppurativa at the knees.

Hidradenitis suppurativa is a poorly understood, destructive disease centered on pilosebaceous units and characterized by inflammatory nodules that progress to abscesses, sinus tracts, and scars. The typical patient is a young woman with involvement of the axillae, breasts, and groin. Presented is a 60-year-old man with poorly controlled diabetes, who initially developed pink-to-yellowish plaques with punched out ulcers on his knees that evolved to scars and draining sinus tracts. Draining sinus tracts were also noted under the left axilla. Histopathology revealed suppurative granulomatous inflammation centered on hair follicles as well as sinus tracts. Special stains for fungus and mycobacterial bacilli were negative. Cultures and PCR for mycobacterial tuberculosis were negative. The patient was diagnosed with hidradenitis suppurativa. A treatment trial of topical corticosteroids and antibiotics was given, but this regimen failed to improve his lesions. The patient was then started on adalimumab, which yielded marked improvement within three months. This case is reported because of the unusual clinical presentation and to highlight the spectrum of atypical hidradenitis suppurativa.

Factors affecting R01 grant funding among academic neurosurgeons over the last decade.

BACKGROUND: Recent studies have reported a gender and medical degree disparity for those receiving Research Project Grants in surgical specialties. The aim of the present study is to analyze factors among academics neurosurgeons that correlate to higher amounts of R01 grant monies awarded. MATERIALS AND METHODS: The National Institutes of Health Research Portfolio Online Reporting Tools Expenditures and Results database was queried for neurosurgery funding between 2008 and 2018. Grant recipients were categorized among type of degree, secondary degree(s), professorship, gender, and h - index. Statistical analysis was performed. RESULTS: The National Institutes of Health awarded 480 R01 grants totaling $182,482,644 to 81 allopathic neurosurgeons between 2008 and 2018. No osteopathic neurosurgeons were awarded an R01 grant during this timeframe. There was a significant difference for type of professorship on the total awarded amount at the p < 0.05 level for the three types of professorship [F (2,78) = 4.85, p < 0.01)]. There was a significant difference for magnitude of h - index on total R01 monies (p < 0.00001). Males accounted for the majority of R01 monies (93.99%); however, no significant difference between average amount awarded and gender was identified (p = 0.86). A secondary degree was without significant difference for R01 amount awarded (p = 0.75). CONCLUSIONS: The present study establishes a medical degree disparity for academic neurosurgeons who receive an R01 grant. Statistically significant factors found to affect amount of R01 grant monies awarded were limited to type of professorship and magnitude of h - index.

A Case of Cachexia Secondary to Obsessive-Compulsive Disorder.

Obsessive-compulsive disorder (OCD), a relatively common psychiatric illness, is diagnosed using DSM-V criteria. Its severity is assessed using the Yale-Brown Obsessive Compulsive Scale (Y-BOCS). Symptoms are broken down into five categories of obsessive-compulsive (O-C) manifestations: contamination/cleaning, symmetry/ordering, taboo thoughts, doubt about harm/checking, and worry about throwing away items that could prove useful or valuable/hoarding. CBT in the form of exposure response therapy (ERP) and/or SSRI/clomipramine administration is the mainstay of treatment. We present a unique OCD case in the nature of obsessions and compulsions, cachexia presentation without anorexia, and history of multiple inpatient psychiatric admissions. Our patient's obsessions focus on eating at specific times, prompting compulsive eating patterns that often result in starvation due to missing timeframes that the patient deems acceptable for eating. His resulting cachexia and eventual worsening of depression to the point of suicidality necessitated multiple inpatient stays and placement at a long-term mental health care facility.

Neurosurgical Management of Lateral Meningocele Syndrome: A Clinical Update for the Pediatric Neurosurgeon.

BACKGROUND: Lateral meningocele syndrome (LMS) is an exceedingly rare connective tissue disease with phenotypic anomalies similar to those seen in Marfan syndrome, Ehler-Danlos syndrome, and Loeys-Dietz syndrome. However, this syndrome is invariably associated with the presence of multiple lateral thoracolumbar spinal meningoceles: a distinct point of phenotypic divergence from other connective tissue disorders. The etiopathogenesis of this syndrome has recently been linked to truncating mutations within exon 33 of NOTCH3. Despite numerous reports, neurosurgical management of multiple spinal meningoceles remains poorly defined in the literature. We conducted a literature review to provide insight into the nosology, clinical significance, and neurosurgical management strategies of this distinct connective tissue disorder. SUMMARY: Our literature search revealed 11 articles (16 cases) of LMS, which included 9 males and 7 females, belonging to 14 different families. Half of these cases underwent genetic screening: all of which were discovered to exhibit a truncating mutation within exon 33 of NOTCH3. All patients exhibited multiple lateral thoracolumbar spinal meningoceles with craniofacial dysmorphisms. Other clinical characteristics included pathologic changes in spine morphology, Chiari I malformation, syringomyelia, hydrocephalus, and tethered cord. Operative management of multiple spinal meningoceles in LMS is complicated by the presence of such coexisting structural neurologic pathologies, which may alter cerebrospinal fluid flow dynamics and, ultimately, impact operative intervention. Key Messages: LMS is an exceedingly rare connective tissue disorder with severe spinal dural involvement. Neurosurgical management of multiple spinal meningoceles is complex, which is further complicated by the presence of coexisting neuropathology, such as pathologic transformation of spine morphology and Chiari I malformation. Patients with a connective tissue disorder phenotype found to have multiple spinal meningoceles on imaging studies may benefit from evaluation by a medical geneticist and a pediatric neurosurgeon.