Type III cutaneous atypical meningioma of the scalp.

Meningiomas occur rarely in extracranial sites, including the skin, where they may pose a diagnostic challenge because of their histopathologic overlap with several other spindle-cell tumors. Cutaneous meningiomas are divided into type I (congenital), type II (ectopic), and type III (via direct extension) lesions. We present a rare case of atypical meningioma of the skin in a 71-year-old woman who presented with a painful and enlarging lesion on the left central frontal scalp. Biopsy showed bone and soft tissue with involvement of a spindle cell neoplasm, consisting of whorled nests with atypical features, including variably increased mitotic index, areas of hypercellularity, and sheeted architecture. The overall findings were consistent with an atypical meningioma (World Health Organization grade 2). Atypical meningiomas constitute only 5% to 15% of all meningiomas. Magnetic resonance imaging of the skull later demonstrated a left frontal tumor consistent with an atypical meningioma that had eroded through the skull. Dermatopathologists should consider cutaneous meningioma as a differential diagnosis of spindle-cell neoplasms of the skin and subcutaneous tissue in head and neck.

Cutaneous atypical fibroxanthoma with osteoclast-like giant cell: A rare phenomenon.

Atypical fibroxanthoma (AFX) with osteoclast-like giant cells is a rare entity. We present the case of an elderly woman who presented with a pink-purple dome-shaped nodule with central hyperkeratotic crust. Biopsy revealed a cellular, dermal-based tumor comprised of spindle, oval, and osteoclast-like giant cells with pleomorphism. The immunohistochemistry profile supported a diagnosis of AFX with osteoclast-like giant cells. We performed a literature review through PubMed and Google Scholar for AFX with osteoclast-like giant cell formation and found 16 previously reported cases. We aim to provide a review and discuss features of these cases. We also discuss the pathogenesis of these osteoclast-like cells as well as potential pitfalls in diagnosis.

Pancreatic Carcinosarcoma Clinical Outcome Analysis of the National Cancer Institute Database.

BACKGROUND: Pancreatic carcinosarcomas (PCS) are rare aggressive biphasic malignancies with a poor prognosis. We aimed to improve the understanding of PCS by analyzing variables that influence the mortality of PCS patients. METHODS: The Surveillance, Epidemiology, and End Results database was queried for cases of PCS from 1973 to 2016. Cases were analyzed for patient demographics, tumor characteristics, and surgical intervention. Kaplan-Meier and Cox regression analyses were applied to investigate the overall survival (OS) and prognostic factors. RESULTS: Thirty-nine cases of PCS were identified along with the disease demographics and characteristics. The majority of patients had a regionally invasive or metastatic disease. There was a significant decrease in OS with the increase of the tumor extension. Conversely, surgery showed to improve OS in the crude analysis, including patients that underwent lymphadenectomy. In addition, the unadjusted Cox regression results showed decreased hazard ratios with a local disease versus distant metastasis and with cancer-directed surgery versus no surgery. Nevertheless, the adjusted Cox regression results revealed that metastatic disease was the only significant predictor of survival. CONCLUSIONS: This population-based study provides some insight to a very rare disease by analyzing 39 cases of PCS. Our finding suggests considering PCS as a nonsurgical disease and reserving surgery solely for patients with a localized disease in combination or after neoadjuvant therapy. Consequently, there is a need to further investigate novel therapies for this aggressive malignancy.

Percutaneous Route of Life: Chylothorax or Total Parenteral Nutrition-Related Bilateral Pleural Effusion in a Neonate?

OBJECTIVE: Peripherally Inserted Central Catheter (PICC) lines are an essential tool in the management of premature neonates. Pleural effusion (PLE) secondary to the leakage of alimentation into the pleural cavity is an encountered complication of central-line total parenteral nutrition (TPN) administration. Methods: We review a case of a premature neonate who suffered large, bilateral PLE after insertion of an upper extremity PICC line for TPN. Results: Pleural fluid biochemical analysis confirmed PICC line infiltration, predominantly with monocytes, low protein, high triglycerides and high glucose. These results favored TPN leakage over chylothorax. Conclusions: To our knowledge, this is the first case of bilateral PLE due to PICC complication in a neonate, which highlights the importance of chylothorax differential diagnosis, the role of autopsy, and the need for clinical precautions when providing premature neonates with high osmolarity TPN.

Impact of partnership status on clinical outcomes of patients with vulvar squamous cell carcinoma and performance of sentinel lymph node biopsy.

OBJECTIVES: Vulvar squamous cell carcinoma is a rare malignancy and lymph node involvement is the most significant prognostic factor. We aimed to evaluate the association between partnership status and mortality from vulvar squamous cell carcinoma, cancer stage at the time of presentation, and the decision for sentinel lymph node biopsy. METHODS: The US National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database was queried and different parameters were evaluated relative to partnership status. A total of 4851 patients with vulvar squamous cell carcinoma, >18 years of age, who presented between January 2010 to December 2015, were analyzed. Kaplan-Meier and Cox regression analyses were used to assess survival and hazard ratio. Multinomial regression analysis and χ2 were utilized to evaluate odd ratios and significance of variables. RESULTS: Most patients were unpartnered (58.5%), including never married (17.7%), divorced (13.8%), or widowed (27%). Partnered patients were mostly Caucasian (88.4%), insured (74%), and presented with stage I disease (57.2%), compared with unpartnered patients (79.1%), (61.7%), and (51.7%), respectively (p<0.01). The mean survival time (months) in partnered patients was longer, compared with unpartnered (p<0.001), and the difference between both groups increased from 9 months at stage I to 24 months at stage IV, which remained independently significant after adjusting the different variables. Cox regression showed that partnered patients had a lower hazard ratio than unpartnered patients (p<0.01). Mortality from vulvar squamous cell carcinoma increased with age at diagnosis, no surgery, and unemployment (p<0.01). Unpartnered patients were the least likely to undergo sentinel lymph node biopsy in early stages, compared with partnered (p<0.01). Univariate Cox regression analysis showed that not performing sentinel lymph node biopsy almost doubled the hazard ratio of vulvar squamous cell carcinoma (p<0.01). CONCLUSIONS: Partnership status should be considered when counseling patients for vulvar squamous cell carcinoma therapy and when recommending screening and follow-up to optimize patient care.

Zebras in Foreskin Dermatopathology: A Review.

OBJECTIVES: The aim of the study was to review uncommon foreskin dermatopathology conditions clinically and pathologically. METHODS: A database search of PubMed and Google Scholar were extracted between March 1, 2009, and March 1, 2019, using the search terms "foreskin," "prepuce," "penis," "pathology," "dermatology," and "rare." The search was limited to "humans" and "dermatopathology." Full article texts were reviewed. Reference lists were screened for additional articles. Patient details (diagnosis, dermatopathology, treatment, and follow-up if available) were extracted. We excluded articles written in the non-English language, unusual variants of common conditions, and cases of common dermatologic conditions. RESULTS: A list of 369 articles was identified and another screening identified 30 articles for rare foreskin pathologies. Those are divided into categories based on the following etiologies: (a) benign, including congenital (e.g., aposthia), infectious (graft versus host disease and histoplasma), autoimmune (Crohn's disease and pyoderma gangrenosum), and benign neoplasms (neurofibroma, apocrine hidrocystoma, verruciform xanthoma, porokeratosis, penile cutaneous horn, localized amyloidosis) and (b) malignancies, including primary (myeloid sarcoma, basal cell carcinoma, Kaposi's sarcoma, mucosal-associated lymphoid tissue lymphoma), and metastasis. CONCLUSIONS: We reviewed and discussed unusual benign and malignant dermatopathology conditions that can affect the foreskin.

Revolutionizing diagnostic pathology: The emergence and impact of artificial intelligence-what doesn't kill you makes you stronger?

This study explored the integration and impact of artificial intelligence (AI) in diagnostic pathology, particularly dermatopathology, assessing its challenges and potential solutions for global health care enhancement. A comprehensive literature search in PubMed and Google Scholar, conducted on March 30, 2023, and using terms related to AI, pathology, and machine learning, yielded 44 relevant publications. These were analyzed under themes including the evolution of deep learning in pathology, AI's role in replacing pathologists, development challenges of diagnostic algorithms, clinical implementation hurdles, strategies for practical application in dermatopathology, and future prospects of AI in this field. The findings highlight AI's transformative potential in pathology, underscore the need for ongoing research, collaboration, and regulatory dialogue, and emphasize the importance of addressing the ethical and practical challenges in AI implementation for improved global health care outcomes.

From endometrium to fingernail bed: histological evaluation of a rare cutaneous metastasis.

In the dermatological spectrum of oncologic manifestations, cutaneous metastases from endometrial carcinoma stand as a rarity, given the tumour's predilection for neighbouring uterine regions. We present an exceptional case of a patient in her mid-50s, whereby an endometrial carcinoma, defying conventional pathways, manifested on the skin and nail of her distal fourth finger, an unusual site for cutaneous metastases, with a specific histology of the primary cancer.

Dupilumab-Associated Sezary Syndrome.

Dupilumab is a monoclonal antibody that inhibits interleukin-4 (IL-4) and interleukin-13 (IL-13), approved for the treatment of adults with moderate-to-severe atopic dermatitis (AD). While recent reports have described cases of new-onset mycosis fungoides (MF) following treatment with dupilumab for AD, to our knowledge only one patient has been delineated with the progression to SS. We present an additional case of a patient who was diagnosed with SS following treatment with dupilumab for adult-onset AD and asthma. We examine SS as a possible side effect of dupilumab while also discussing management and theories to explain this phenomenon.

To treat or not to treat: PD-L1 inhibitor-induced keratoacanthoma and squamous cell carcinoma.

Keratoacanthoma (KA) and squamous cell carcinoma (SCC) are rare side effects of programmed cell death ligand-1 (PD-L1) inhibitors that can disrupt therapy. There is no consensus on optimal treatment. We investigated the management strategy and factors influencing pathophysiology. An institutional cancer registry and literature search were used for this retrospective study. Only PD-L1-induced KA and SCC cases were included. Pathology specimens were stained with immune markers and management strategies were analyzed. Four cases were identified at our institution. Immunohistochemistry of atypical keratinocytes revealed PD-1/PD-L1 positivity, high p53, and low bcl-2 for all cases with differential expression of CD44 and beta-catenin for KA versus SCC. Nivolumab was continued or temporarily held with complete resolution. In addition, a literature search identified 30 additional cases of KA/SCC after PDL-1 inhibitor use. The most common treatment was excision/destruction followed by topical and/or intralesional corticosteroids. Therapy was definitely withheld in 22% of KA patients and in 9% of SCC cases. The expression of PD-L1 by atypical keratinocytes helps to explain the effects of nivolumab on the development of cutaneous neoplasms. The expression of immune markers provides mechanistic insights into pathophysiology. Management may be achieved with conservative therapy and without treatment interruption.

Dupilumab-associated mycosis fungoides: a cross-sectional study.

Treating atopic dermatitis (AD) with dupilumab, a monoclonal antibody that inhibits interleukin-4 (IL-4) and interleukin-13 (IL-13), may be associated with the progression of mycosis fungoides (MF).This study aims to examine the associations between the length of dupilumab treatment, age and sex, and the onset of MF.An institutional data registry and literature search were used for a retrospective cross-sectional study. Only patients with a diagnosis of MF on dupilumab for the treatment of AD and eczematous dermatitis were included.The primary outcome was the length of dupilumab exposure, age, sex, and the onset of MF. Linear correlations (Pearson) and Cox regression analysis were used to assess the correlation and the risk.A total of 25 patients were included in this study. Five eligible patients were identified at our institution. In addition, a PubMed review identified an additional 20 patients. At the time of MF diagnosis, the median age was 58, with 42% female. Disease history was significant for adult-onset AD in most patients (n = 17, 65.4%) or recent flare of AD previously in remission (n = 3, 11.5%). All patients were diagnosed with MF, and one patient progressed to Sézary syndrome while on dupilumab, with an average duration of 13.5 months of therapy prior to diagnosis. Tumor stage at diagnosis of MF was described in 19 of the cases and ranged from an early-stage disease (IA) to advanced disease (IV). Treatment strategies included narrow-band UVB therapy, topical corticosteroids, brentuximab, pralatrexate, and acitretin. Male gender, advanced-stage disease, and older age correlated significantly with the hazard of MF onset and a shorter time to onset during dupilumab treatment.Our results suggest a correlation between the duration of dupilumab treatment and the diagnosis of MF, the higher MF stage at diagnosis, and the shorter the duration of using dupilumab to MF onset. Furthermore, elderly male patients appeared to be more at risk as both male gender and older age correlated with a hazard of MF diagnosis. The results raise the question as to whether the patients had MF misdiagnosed as AD that was unmasked by dupilumab or if MF truly is an adverse effect of treatment with dupilumab. Close monitoring of these patients and further investigation of the relationship between dupilumab and MF can shed more light on this question .

Case Presentation: Monkeypox.

Monkeypox is a rare viral disease that initially appeared in West and Central Africa, and is primarily transmitted to humans from animals. It moved to the United States in 2003, which was the first time the disease had left the African continent. It is still uncommon in the United States and elsewhere, although outbreaks have occurred, especially in gay communities,1-3 causing it to be declared a health emergency in some areas. (SKINmed. 2022;20:410-411).

What we have learned from the COVID-19 pandemic: Time to think outside the box, maybe far outside.

The coronavirus disease 2019 pandemic has had a profound effect on our lives and careers; this presentation explores some of the lessons we have learned from it and others that it may yet teach us. Socioeconomic effects have been profound, not all of them favorable. Travel and meeting activities, as well as many other activities, have been severely restricted. Social unrest has become intense, and it may have questionable political consequences, as the United States is undergoing a contested election result.

Long-Term Recurrence of Retroperitoneal Dedifferentiated Liposarcoma After a Complete Surgical Resection: A Report of a Rare Phenomenon.

The objective of this report is to present a rare case of a recurrence after 20 years of retroperitoneal dedifferentiated liposarcoma after surgical resection and to discuss the lessons learned from this rare phenomenon for patients management and understanding the behavior of these aggressive tumors.  A 75-year-old woman presented with recurrent retroperitoneal dedifferentiated liposarcoma who had undergone a surgical resection 20 years earlier and had no evidence of disease on frequent follow-ups during that period. The histopathologic examination revealed different morphologic characteristics between the initial and recurrent presentations. The fluorescence in situ hybridization showed amplification of the mouse double minute 2 homolog (MDM2), a regulator of p53 gene on chromosome 12q15, and positive cyclin-dependent kinase 4 (CDK4) immunostain. Liposarcoma long-term recurrence is a challenging surgical disease to provide the best survival outcome. Incomplete resection could explain the recurrence in anatomic locations where the lesions are intermixed with the neighboring adipose tissue. However, dedifferentiated liposarcoma can rarely recur after 20 years. The molecular transformation and the survival analysis of these tumors predict certain behaviors. The refraction for radiation therapy in our case and the mixed morphology provide some insight into the biology and the clinical management for these aggressive tumors.

"Do-not-resuscitate (DNR)" status determines mortality in patients with COVID-19.

We investigatd the influence of do-not-resuscitate (DNR) status on mortality of hospital inpatients who died of COVID-19. This is a retrospective, observational cohort study of all patients admitted to two New Jersey hospitals between March 15 and May 15, 2020, who had, or developed, COVID-19 (1270 patients). Of these, 640 patients died (570 [89.1%] with and 70 [10.9%] without a DNR order at the time of admission) and 630 survived (180 [28.6%] with and 450 [71.4%] without a DNR order when admitted). Among the 120 patients without COVID-19 who died during this interval, 110 (91.7%) had a DNR order when admitted. Deceased positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) patients were significantly more likely to have a DNR order on admission compared with recovered positive SARS-CoV-2 patients (P < 0.05), similar to those who tested negative for SARS-CoV-2. COVID-19 DNR patients had a higher mortality compared with COVID-19 non-DNR patients (log rank P < 0.001). DNR patients had a significantly increased hazard ratio of dying (HR 2.2 [1.5-3.2], P < 0.001) compared with non-DNR patients, a finding that remained significant in the multivariate model. The risk of death from COVID-19 was significantly influenced by the patients' DNR status.

Granuloma Annulare - A Manifestation of Primary Cutaneous Mucinous Carcinoma?

The objective of this report is to present a rare case of primary cutaneous mucinous carcinoma (PCMC) manifesting with granuloma annulare (GA), and to discuss the association as a paraneoplastic phenomenon. A 65-year-old female presented with a painless, slow-growing, cystic nodule less than 1 cm over the left lateral canthus. The clinical presentation was highly suspicious of sebaceous cyst. The histopathologic examination revealed variable sizes of neoplastic cell clusters in a pool of abundant mucin. A focus of palisading lympho-histiocytic infiltrate surrounding a necrobiosis suggestive of granuloma annulare adjacent to the tumor is identified. Series of extensive investigations performed did not reveal any primary origin. GA can rarely be associated with various malignant conditions. Its association and prognostic importance to these conditions are unclear. The presence with certain malignancies and the resolution of GA with the treatment of underlying malignancy are an indicator that this condition can very well be a paraneoplastic phenomenon.

A Rare Mimic of Malignancy: Papillary Endosalpingiosis.

Endosalpingiosis is the presence of ectopic Müllerian-type epithelium resembling Fallopian tube epithelium. It usually appears as small glandular inclusions in various peritoneal sites or lymph nodes. Rarely, it is papillary in configuration, and lack of familiarity with this entity could lead to overinterpretation as a malignancy. We recently encountered a case of papillary endosalpingiosis found incidentally at laparotomy for ectopic pregnancy. Awareness of this entity is important, as it may be a diagnostic challenge.

Correction to: Treatment of Rare and Aggressive Pancreatic Carcinosarcoma.

[This corrects the article on p. e00379 in vol. 7, PMID: 32607379.].

Primary Cutaneous Clear Cell Sarcoma, Clinical Outcome With Sentinel Lymph Nodes Status.

OBJECTIVES: Primary cutaneous clear cell sarcoma (PCS) is a rare malignancy and difficult to differentiate from melanoma. We investigated factors influencing survival and recurrence. METHODS: An institutional cancer registry and literature search were used for a retrospective study. Only clear cell sarcoma cases with a primary site of skin and subcutaneous tissue were included. Kaplan-Meier and Cox regression analyses were used to assess survival time and hazard ratios. RESULTS: Three eligible cases were identified at our institution. In addition, the PubMed and Google Scholar reviews identified 1,878 items, with 23 patients with PCS. The median age was 25 years with 62% female. The tumors ranged in size from 0.4 to 4.5 cm. Cytogenetics showed t(12;22)(q13;q12) in all cases and a unique variant of t(2;22)(q32.3;q12) in one case. Surgery was the most common treatment, followed by chemotherapy/radiation. PCS recurred in 46% of patients with a median relapse-free survival time of 15 months. Only two known PCS-related mortalities were recorded, at 38 and 60 months following initial diagnosis. Smaller tumor size and negative sentinel lymph node biopsy (SLNB) status were significantly associated with a better disease-free survival. CONCLUSIONS: Tumor size and SLNB status influence PCS survival and recurrence. More research is needed due to the rarity of this disease.