Risk factors for recurrent disease after resection of solitary fibrous tumor: a systematic review.

Introduction: Solitary fibrous tumor (SFT) is a rare soft tissue tumor found at any site of the body. The treatment of choice is surgical resection, though 10%-30% of patients experience recurrent disease. Multiple risk factors and risk stratification systems have been investigated to predict which patients are at risk of recurrence. The main goal of this systematic review is to create an up-to-date systematic overview of risk factors and risk stratification systems predicting recurrence for patients with surgically resected SFT within torso and extremities. Method: We prepared the review following the updated Prisma guidelines for systematic reviews (PRISMA-P). Pubmed, Embase, Cochrane Library, WHO international trial registry platform and ClinicalTrials.gov were systematically searched up to December 2022. All English studies describing risk factors for recurrence after resected SFT were included. We excluded SFT in the central nervous system and the oto-rhino-laryngology region. Results: Eighty-one retrospective studies were identified. Different risk factors including age, symptoms, sex, resection margins, anatomic location, mitotic index, pleomorphism, hypercellularity, necrosis, size, dedifferentiation, CD-34 expression, Ki67 index and TP53-expression, APAF1-inactivation, TERT promoter mutation and NAB2::STAT6 fusion variants were investigated in a narrative manner. We found that high mitotic index, Ki67 index and presence of necrosis increased the risk of recurrence after surgically resected SFT, whereas other factors had more varying prognostic value. We also summarized the currently available different risk stratification systems, and found eight different systems with a varying degree of ability to stratify patients into low, intermediate or high recurrence risk. Conclusion: Mitotic index, necrosis and Ki67 index are the most solid risk factors for recurrence. TERT promoter mutation seems a promising component in future risk stratification models. The Demicco risk stratification system is the most validated and widely used, however the G-score model may appear to be superior due to longer follow-up time. Systematic Review Registration: CRD42023421358.

Atypical fibroxanthoma and pleomorphic dermal sarcoma: Local recurrence and metastasis in a nationwide population-based cohort of 1118 patients.

BACKGROUND: The prognosis of patients with atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) remains uncertain and no standardized follow-up programs have been established. OBJECTIVE: To recommend a standardized follow-up program of patients with AFX and PDS based on nationwide long-term estimates of local recurrence and metastasis. METHODS: All patients with AFX and PDS in Denmark between 2002 and 2022 were included. Danish National Registries were used to estimate the risks of local recurrence and metastasis for AFX and PDS. RESULTS: The 5-year risk of local recurrence was 10% for AFX and 17% for PDS. The 5-year risk of metastasis was 0.8% for AFX and 16% for PDS. PDS metastasized within 3 years in >90% of the patients with the lungs as the primary metastasis site (50%). Invasion beyond the subcutis, perineural/intravascular infiltration, and increasing age significantly increased the risk of PDS relapse. LIMITATIONS: Risk of misclassification and lack of detailed surgical information. CONCLUSION: The follow-up of patients with AFX can be limited to clinical visits for 4 years. Patients with PDS should be followed with clinical visits and PET/CT twice a year for the first 3 years and once a year for a minimum of 1 year.

Cutaneous malakoplakia.

Malakoplakia is a form of chronic, granulomatous, inflammatory condition which usually affects the genitourinary tract or other internal organs of immunocompromised patients. It is usually caused by acquired bactericidal incapacity of macrophages in connection to Eschericia coli infection. This case report presents an extremely rare case of cutaneous malakoplakia in the left axilla of a 48-year-old male patient, who had undergone kidney transplant one year earlier. The clinical presentation of cutaneous malakoplakia varies from nodules to plaques and moist wounds. The primary treatment is long-term antibiotic therapy.

Immunohistochemical Characteristics of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: A Systematic Review and Meta-Analysis.

BACKGROUND: Differentiating atypical fibroxanthoma (AFX) from pleomorphic dermal sarcoma (PDS) remains a challenge. Increasing the use of immunohistochemistry has led to the proposal of many immunomarkers that may aid in the diagnosis of AFX and PDS. In this meta-analysis, we investigate the immunohistochemical characteristics of AFX and PDS based on suggested immunomarkers in the literature. Second, we identify potential distinctive markers found in the tumors' respective immunohistochemical profiles. METHODS: We included studies using immunomarkers on at least 10 consecutive patients with clinically and histopathologically verified AFX or PDS. The positive rates of the immunomarkers were pooled across the included studies with random-effects models. The immunomarkers were further categorized by a priori-chosen cutoffs in positive rates as positive markers (>90%) or negative markers (<10%). Differences between AFX and PDS were compared with Wald tests. RESULTS: We included 45 studies (1516 tumors) reporting on 35 immunomarkers. CD10 was positive in 94% (95% confidence interval, 87-99) of AFX cases and 100% (95% confidence interval, 99-100) of PDS cases. In accordance with the literature, both AFX and PDS were mainly negative for epithelial markers, melanocytic markers, markers of smooth muscle differentiation, and endothelial markers. None of the examined immunomarkers could distinguish AFX from PDS. CONCLUSIONS: Our results suggest that CD10 is a useful positive immunomarker for both AFX and PDS. We found no difference in immunohistochemical profile when comparing AFX with PDS. Our analysis suggests that CD10, AE1/AE3, CK5/CK6, p63, S100, SOX10, desmin, SMA, CD31, and ERG could be used to differentiate AFX and PDS from other spindle cell neoplasms.

Radiation Induced Atypical Vascular Lesion in the Breast.

BACKGROUND AND PURPOSE: Atypical vascular lesion (AVL) became a separate WHO diagnosis in November 2019. Due to a possible risk of developing angiosarcoma, extensive surgery with excision of AVL has been recommended but the benefit from this is questionable. We investigated whether the change in WHO classification has led to an increase in the number of patients diagnosed with AVL, thereby leading to an increase in extensive surgery. METHOD: The Danish National Pathology Databank was used to identify patients diagnosed with AVL between June 1, 2010 to June 31, 2020. The rate of AVL diagnosed before and after change in WHO classification was compared. RESULTS: In total, 13 cases of AVL were identified, 3 cases diagnosed before changes in WHO classification corresponding to 0.025 cases per month, compared to 8 cases, 1.143 cases per month, after the change in WHO classification. This corresponded to a 45-fold increase (95%CI: 10.88-265,31) (P < .0001) in AVL diagnosis. The mean patient age at diagnosis was 67 years. Patients received treatment varying from yearly follow up to extensive surgery. Non developed angiosarcoma in the follow-up period of 22 months. CONCLUSION: The changes in WHO classification of AVL has led to a considerable increase in the number of patients diagnosed with the lesion. No standardized treatment exists for this rare condition, but extensive surgery is often recommended to this frail population despite the lack of evidence for prognostic benefit from the procedure. Prospective follow-up studies are needed to determine the optimal treatment strategy.

Case Report: Giant Paraganglioma of the Skull Base With Two Somatic Mutations in SDHB and PTEN Genes.

Head and neck paragangliomas (HNPGLs) are neuroendocrine tumors. They arise from the parasympathetic ganglia and can be either sporadic or due to hereditary syndromes (up to 40%). Most HNPGLs do not produce significant amounts of catecholamines. We report a case of a giant paraganglioma of the skull base with an unusually severe presentation secondary to excessive release of norepinephrine, with a good outcome considering the severity of disease. A 39-year-old Caucasian woman with no prior medical history was found unconscious and emaciated in her home. In the intensive care unit (ICU) the patient was treated for multi-organ failure with multiple complications and difficulties in stabilizing her blood pressure with values up to 246/146 mmHg. She was hospitalized in the ICU for 72 days and on the 31st day clinical assessment revealed jugular foramen syndrome and paralysis of the right n. facialis. A brain MRI confirmed a right-sided tumor of the skull base of 93.553 cm3. Blood tests showed high amounts of normetanephrine (35.1-45.4 nmol/L, ref <1.09 nmol/L) and a tumor biopsy confirmed the diagnosis of a paraganglioma. Phenoxybenzamine and Labetalol were used in high doses ((Dibenyline®, 90 mg x 3 daily) and labetalol (Trandate®, 200 + 300 + 300 mg daily) to stabilize blood pressure. The patient underwent two tumor embolization procedures before total tumor resection on day 243. Normetanephrine and blood pressure normalized after surgery (0.77 nmol/L, ref: < 1.09 nmol/L). The damage to the cranial nerve was permanent. Our patient was comprehensively examined for germline predisposition to PPGLs, however we did not identify any causal aberrations. A somatic deletion and loss of heterozygosity (LOH) of the short arm (p) of chromosome 1 (including SDHB) and p of chromosome 11 was found. Analysis showed an SDHB (c.565T>G, p.C189G) and PTEN (c.834C>G, p.F278L) missense mutation in tumor DNA. The patient made a remarkable recovery except for neurological deficits after intensive multidisciplinary treatment and rehabilitation. This case demonstrates the necessity for an early tertiary center approach with a multidisciplinary expert team and highlights the efficacy of the correct treatment with alpha-blockade.

Extra-axial chordoma of the thumb: Report of a rare case with clinicopathologic and molecular analysis.

Chordoma is a very rare malignant tumor, with a phenotype that recapitulates notochord, and is chiefly located in the axial skeleton with only few cases reported in the extra-axial skeleton and soft tissues. The diagnosis can be challenging for both clinicians, radiologists and pathologists because of the rarity of tumor, its unspecific radiological pattern and histomorphological similarities to other tumors like extra-skeletal myxoid chondrosarcoma, soft tissue myoepithelioma and metastatic adenocarcinomas, more so on small biopsies. We present a case of a recurrent extra-axial chordoma with a prominent soft tissue component in the left thumb around proximal phalanx of an 80-year-old man, with detailed report of the histopathological, imaging and most importantly molecular features, which are in conformity with the typical profile of notochordal neoplasms. To the best of our knowledge, we report the first DNA-methylation- and the copy number variation analysis of an extra-axial chordoma with a very rare localization, thumb. With this case study we try to give a better understanding of tumor's specification, lessen the diagnostic confusion by highlighting its extra-axial occurrence, and more importantly present substantial molecular data, which might help in providing more therapeutic opportunities in the future.

Basal Cell Carcinoma of Prostate With MSMB-NCOA4 Fusion and a Probable Basal Cell Carcinoma In Situ: Case Report.

Basal cell carcinomas of prostate (BCCP) are very rare. Most arise in the transition zone and thus are associated with lower urinary tract symptoms and rarely associated with elevated prostate-specific antigen (PSA). These features make diagnosis/early diagnosis difficult because of the routine protocols followed. Basal cell carcinomas have distinctive histopathological, immunohistochemical, and to some extent also different molecular characteristics. Basal cell carcinoma in situ (BCCIS) is a nonexistent histological lesion as per the current literature, but here is an attempt to describe it through this case.A 74-year-old man presented with hematuria and previous diagnosis of prostatic hyperplasia. Based on this history, he underwent a prostatectomy ad modum Freyer. Pathological examination surprisingly revealed a diffusely infiltrative tumor with nonacinar adenocarcinoma morphology and many glandular structures probably representing BCCIS. Tumor was diagnosed as BCCP. Patient presented with metastasis to the abdominal wall 8 months postprostatectomy.BCCP is an aggressive type of prostate cancer, which might be challenging to diagnose based on routine protocols. This results in delayed diagnosis and treatment and thus poor prognosis. Furthermore, patients with this subtype of prostate cancer need appropriately designed, and maybe a totally different follow-up regimen as PSA is of no use for BCCP patients. Finally, diagnosis of BCCIS, if agreed upon its existence needs to be studied in larger cohorts as a precursor lesion.

Sarcomas with sclerotic epithelioid phenotype harboring novel EWSR1-SSX1 fusions.

Undifferentiated sarcomas remain difficult to classify. Despite the remarkable advances in sarcoma classification made by the increased application of RNA sequencing in clinical practice, the unexpected result of a novel gene fusion raises further questions regarding the tumor histogenesis and subclassification. In this study, we present two high grade sarcomas with epithelioid phenotype occurring in the deep-soft tissues (shoulder, thigh) of young adults which based on the non-specific pathologic findings were deemed unclassified and subjected to targeted RNA sequencing for further diagnostic interpretation. The results showed an identical EWSR1 exon 7-SSX1 exon 5 fusion. The breakpoints in both genes represent similar hot spots as seen in Ewing sarcoma and synovial sarcoma, generating a fusion transcript predicted to be in frame, and to retain the same protein domains within the fusion oncoprotein. These results were further confirmed by FISH analysis for both break-apart and fusion come-together assays in both genes. Both tumors showed a round to epithelioid morphology associated with extensive stromal hyalinization and necrosis. One case showed scattered psammomatous calcifications. The tumors shared a similar immunoprofile, including reactivity for EMA, CK, TLE1, BCOR, and CD99, while negative for S100, SOX10, CD34, SMA, and desmin. Both cases showed MUC4 positivity (one diffuse, one patchy), while one case showed patchy ALK positivity. One patient developed lymph node metastases, while the other showed no evidence of disease at 6-month follow-up. Neither case fit in any known pathologic categories. Larger series are needed to interrogate if the presence of EWSR1-SSX1 fusion defines a novel pathologic entity of a sarcoma with epithelioid cytomorphology, sclerotic stroma, and epithelial differentiation immunohistochemically.

Preoperative Embolization of a Solitary Fibrous Tumor Originating from External Auditory Meatus: A Case Report with Literature Review.

Solitary fibrous tumors (SFTs) are mesenchymal, fibroblastic tumors with mostly favorable, but still unpredictable prognosis. Their rarity and occurrence at a variety of locations coupled with variable histological appearance make the diagnosis a challenge. This can be resolved by histological and immunohistochemical analysis on the histologic material eventually coupled with demonstration of NAB2-STAT6 gene fusion by next generation sequencing (NGS) analysis. Tumor removal with clear surgical margins is sufficient for complete cure in most cases. Percutaneous transcatheter embolization in well-vascularized lesions may minimize the risk of bleeding during subsequent removal. In this article we present a rare case of SFT arising from the external auditory canal and treated with preoperative endovascular arterial embolization. A literature review with focus on diagnostics and treatment of this entity in the head and neck region is following.

Soft tissue myoepithelial carcinoma-a case report with molecular and electron microscopic analysis.

Myoepithelial tumors are rare neoplasms that primarily occur in the salivary gland but over the last decade have also been described in other locations, including soft tissue. Myoepithelial carcinoma of soft tissue has shown to have a recurrence rate of 39-42% and distant metastases in 32-53% of patients. The only known predictor of malignancy in soft tissue myoepithelial tumors described is cytologic atypia. There are no other validated criteria for differentiating benign and malignant tumors, neither for grading nor for standard of care for these tumor types. Herein, we present a case of myoepithelial carcinoma of inguinal soft tissue in a 37-year male. We also describe the molecular and previously sparingly described detailed electron microscopic features of this case.

In vitro 4-1BB stimulation promotes expansion of CD8+ tumor-infiltrating lymphocytes from various sarcoma subtypes.

Tumor-specific tumor-infiltrating lymphocytes (TILs) can be in vitro expanded and have the ability to induce complete and durable tumor regression in some patients with melanoma following adoptive cell therapy (ACT). In this preclinical study, we investigated the feasibility of expanding TIL from sarcomas, as well as performing functional in vitro analyses on these. TILs were expanded in vitro by the use of IL2 stimulation with or without the addition of 4-1BB and CD3 antibodies. Phenotypical and functional analyses were mainly performed by flow cytometry. TILs were expanded from 25 of 28 (89%) tumor samples from patients with 9 different sarcoma subtypes. TILs were predominantly αß T-cells of effector memory subtype with CD4+ dominance. In particular, CD8+ TIL highly expressed LAG3 and to a lesser degree PD-1 and BTLA. In total, 10 of 20 TIL cultures demonstrated in vitro recognition of autologous tumor. In some cases, the fraction of tumor-reactive T cells was more than 20%. 4-1BB stimulation augmented expansion kinetics and favored CD8+ occurrence. In conclusion, TIL expansion from sarcoma is feasible and expanded TILs highly express LAG3 and comprise multifunctional tumor-reactive T-cells.

Metabolic control of PPAR activity by aldehyde dehydrogenase regulates invasive cell behavior and predicts survival in hepatocellular and renal clear cell carcinoma.

BACKGROUND: Changes in cellular metabolism are now recognized as potential drivers of cancer development, rather than as secondary consequences of disease. Here, we explore the mechanism by which metabolic changes dependent on aldehyde dehydrogenase impact cancer development. METHODS: ALDH7A1 was identified as a potential cancer gene using a Drosophila in vivo metastasis model. The role of the human ortholog was examined using RNA interference in cell-based assays of cell migration and invasion. 1H-NMR metabolite profiling was used to identify metabolic changes in ALDH7A1-depleted cells. Publically available cancer gene expression data was interrogated to identify a gene-expression signature associated with depletion of ALDH7A1. Computational pathway and gene set enrichment analysis was used to identify signaling pathways and cellular processes that were correlated with reduced ALDH7A1 expression in cancer. A variety of statistical tests used to evaluate these analyses are described in detail in the methods section. Immunohistochemistry was used to assess ALDH7A1 expression in tissue samples from cancer patients. RESULTS: Depletion of ALDH7A1 increased cellular migration and invasiveness in vitro. Depletion of ALDH7A1 led to reduced levels of metabolites identified as ligands for Peroxisome proliferator-activated receptor (PPARα). Analysis of publically available cancer gene expression data revealed that ALDH7A1 mRNA levels were reduced in many human cancers, and that this correlated with poor survival in kidney and liver cancer patients. Using pathway and gene set enrichment analysis, we establish a correlation between low ALDH7A1 levels, reduced PPAR signaling and reduced patient survival. Metabolic profiling showed that endogenous PPARα ligands were reduced in ALDH7A1-depleted cells. ALDH7A1-depletion led to reduced PPAR transcriptional activity. Treatment with a PPARα agonist restored normal cellular behavior. Low ALDH7A1 protein levels correlated with poor clinical outcome in hepatocellular and renal clear cell carcinoma patients. CONCLUSIONS: We provide evidence that low ALDH7A1 expression is a useful prognostic marker of poor clinical outcome for hepatocellular and renal clear cell carcinomas and hypothesize that patients with low ALDH7A1 might benefit from therapeutic approaches addressing PPARα activity.

An updated review on primary signet-ring cell carcinoma of the urinary bladder and report of a case.

OBJECTIVE: The aim of the present study was to emphasize the critical importance of the clinician's awareness of signet-ring cell carcinoma (SRCC) of the urinary bladder, a rare and aggressive disease entity. MATERIALS AND METHODS: A review of the current literature was conducted and a classic case of advanced SRCC of the urinary bladder is reported, clearly demonstrating the severity of this disease and the imperative need for standardized recommendations for the diagnostic work-up and management of urinary bladder SRCC. RESULTS: The prognosis for patients with SRCC of the urinary bladder is poor, attributed to presentation at advanced stages following asymptomatic progression, inefficacy of multimodality therapy and possibly an aggressive underlying biological phenotype. Treatment options are limited and not well studied. CONCLUSION: Given the rarity of SRCC, multi-institutional clinical trials and international cooperation are mandatory to improve survival for patients with primary SRCC of the bladder.

Germ Cell Neoplasia in Situ and Preserved Fertility Despite Suppressed Gonadotropins in a Patient With Testotoxicosis.

Context: Testotoxicosis is an autosomal-dominant, male-limited disorder. Activating mutations in the luteinizing hormone receptor gene (LHCGR) cause high autonomous testosterone secretion, resulting in early-onset peripheral precocious puberty. Little is known about long-term consequences of testotoxicosis. Case Description: We present a rare case of a patient followed for 25 years with two remarkable outcomes: preserved fertility and germ cell neoplasia in situ (GCNIS). He presented with precocious puberty at 10 months of age and was diagnosed with testotoxicosis due to a de novo heterozygous Asp578Tyr mutation in LHCGR. Testicular biopsy in childhood showed Leydig cell hyperplasia with altered cell maturation. From infancy throughout adulthood, elevated testosterone and estradiol, low inhibin B and anti-Müllerian hormone, and completely suppressed follicle-stimulating hormone and luteinizing hormone were noted. Height acceleration and advanced bone age resulted in a reduced final height. Semen analysis revealed ongoing spermatogenesis, and the patient fathered a child by natural conception. Ketoconazole treatment decreased circulating testosterone in childhood, supported by experimental suppression of testosterone production in his adult testis tissue cultured ex vivo. At 25 years of age, ultrasound revealed a testicular tumor, identified as a Leydig cell adenoma, but unexpectedly with GCNIS present in adjacent seminiferous tubules. Conclusion: The case illustrates that absence of gonadotropins but high intratesticular testosterone concentration is sufficient for spermatogenesis and to allow fatherhood. Our study is also the first description, to our knowledge, of GCNIS in a patient with testotoxicosis. We recommend regular clinical examination and ultrasonic evaluation of the testes in these patients due to potential increased risk of malignancy.

USP21 regulates Hippo pathway activity by mediating MARK protein turnover.

The Hippo pathway, which acts to repress the activity of YAP and TAZ trancriptional co-activators, serve as a barrier for oncogenic transformation. Unlike other oncoproteins, YAP and TAZ are rarely activated by mutations or amplified in cancer. However, elevated YAP/TAZ activity is frequently observed in cancer and often correlates with worse survival. The activity and stability of Hippo pathway components, including YAP/TAZ, AMOT and LATS1/2, are regulated by ubiquitin-mediated protein degradation. Aberrant expression of ubiquitin ligase complexes that regulate the turnover of Hippo components and deubiquitylating enzymes that counteract these ubiquitin ligases have been implicated in human cancer. Here we identify the USP21 deubiquitylating enzyme as a novel regulator of Hippo pathway activity. We provide evidence that USP21 regulates YAP/TAZ activity by controlling the stability of MARK kinases, which promote Hippo signaling. Low expression of USP21 in early stage renal clear cell carcinoma suggests that USP21 may be a useful biomarker.

Deubiquitylating enzyme USP9x regulates hippo pathway activity by controlling angiomotin protein turnover.

The Hippo pathway has been identified as a key barrier for tumorigenesis, acting through downregulation of YAP/TAZ activity. Elevated YAP/TAZ activity has been documented in many human cancers. Ubiquitylation has been shown to play a key role in regulating YAP/TAZ activity through downregulation of a number of Hippo pathway components. Several ubiquitin ligase complexes have been implicated in this process, however, little is known about the deubiquitylating enzymes that counteract these activities to regulate YAP/TAZ. Here we identify the deubiquitylating enzyme USP9x as a regulator of YAP/TAZ activity. We demonstrate that USPx regulates ubiquitin-mediated turnover of the YAP inhibitor, Angiomotin. USP9x acts to deubiquitylate Angiomotin at lysine 496, resulting in stabilization of Angiomotin and lower YAP/TAZ activity. USP9x mRNA levels were reduced in several cancers. Clinically, USP9x mRNA levels were reduced in several cancers with low USPx expression correlating with poor prognosis in renal clear cell carcinoma. Our data indicate that USP9x may be a useful biomarker for renal clear cell carcinoma.

Consultation on urological specimens from referred cancer patients using real-time digital microscopy: Optimizing the workflow.

INTRODUCTION: Centralization of cancer treatment entails a reassessment of the diagnostic tissue specimens. Packaging and shipment of glass slides from the local to the central pathology unit means that the standard procedure is time-consuming and that it is difficult to comply with governmental requirements. The aim was to evaluate whether real-time digital microscopy for urological cancer specimens during the primary diagnostic process can replace subsequent physical slide referral and reassessment without compromising diagnostic safety. METHODS: From May to October 2014, tissue specimens from 130 patients with urological cancer received at Næstved Hospital's Pathology Department, and expected to be referred for further treatment at cancer unit of a university hospital, were diagnosed using standard light microscopy. In the event of diagnostic uncertainty, the VisionTek digital microscope (Sakura Finetek) was employed. The Pathology Department at Næstved Hospital was equipped with a digital microscope and three consultant pathologists were stationed at Rigshospitalet with workstations optimized for digital microscopy. Representative slides for each case were selected for consultation and live digital consultation took place over the telephone using remote access software. Time of start and finish for each case was logged. For the physically referred cases, time from arrival to sign-out was logged in the national pathology information system, and time spent on microscopy and reporting was noted manually. Diagnosis, number of involved biopsies, grade, and stage were compared between digital microscopy and conventional microscopy. RESULTS: Complete data were available for all 130 cases. Standard procedure with referral of urological cancer specimens took a mean of 8 min 56 s for microscopy, reporting and sign-out per case. For live digital consultations, a mean of 18 min 37 s was spent on each consultation with 4 min 43 s for each case, depending on the number of digital slides included. Only in two cases could a consensus regarding the diagnosis not be reached during live consultation; this did not, it should be noted, affect patient treatment. Complete agreement between conventional and digital histopathology diagnosis was reached in all the 53 patients referred to central pathology units. The participating pathologists were in general comfortable using live digital microscopy, but they emphasized that a fast internet connection was essential for a smooth consultation. DISCUSSION AND CONCLUSION: An almost perfect agreement between live digital and conventional microscopy was observed in this study. Live digital consultation allowed cases to be referred from local hospitals to central cancer units without the standard delay caused by shipment. Only a few preselected specimen slides for each patient were presented in live consultation, which reduced the time spent on diagnosis compared to using the conventional method. Implementation of real-time digital microscopy would result in quicker turnaround and patient referral time, and with careful selection of relevant specimen slides for consultation, diagnostic safety would not be compromised.

Cytologic features of primary malignant tumors of skin and adnexae.

OBJECTIVE: To highlight the cytologic features of the cases seen in our institute. STUDY DESIGN: Cases of primary malignant tumors of skin and adnexae diagnosed on cytology with histopathology confirmation were retrieved from case records of 1998-2005. Clinical presentation was noted in all the cases. RESULTS: Thirty primary malignant tumors of skin and adnexae were analyzed. Melanoma was the most common (n=12), followed by squamous cell carcinoma (SCC) (n=5). There were 3 basal cell carcinomas and 2 cases each of sebaceous carcinoma, Paget's disease of the breast and lymphoma. There were single cases of eccrine carcinoma, malignant trichilemmal tumor, undifferentiated carcinoma and extramedullary myeloid cell tumor. All the cases of hemopoietic tumors (n=3) and the majority of the cases (4 of 7) of adnexal were seen to arise from head and neck region. Marjolin's ulcer was seen in 4 of 5 SCCs. Scrape smears were obtained from 8 patients and fine needle aspiration in 22 patients. CONCLUSION: Cytodiagnosis of primary malignant tumors of skin and adnexae is possible based on morphology and clinical presentation.

Lymph node metastasis of soft tissue tumors: a cytomorphologic study.

OBJECTIVE: To study the frequency of regional lymph node metastasis of soft tissue tumors (STT) and to evaluate the utility of fine needle aspiration cytology (FNAC) as an initial investigative modality. STUDY DESIGN: A prospective and retrospective study of over 6 years (1998-2004) was performed to look for frequency of STT metastasizing to lymph nodes. FNAC of enlarged nodes was performed as a routine outpatient procedure after obtaining complete clinical details. Histopathology and immunohistochemistry were correlated where available. RESULTS: Lymph node enlargement was seen in 23 of 241 patients with STTs, of which 19 cases showed involvement (7.88%), synchronous with primary in 12 cases and metachronous in 7 cases. The most common sites of primary tumor were the lower extremity and head and neck region with involved regional lymph nodes. STTs commonly involving lymph nodes were rhabdomyosarcoma and extraskeletal Ewing's/primitive neuroectodermal tumor (PNET); other rare tumors included malignant granular cell tumor, epithelioid hemangioendothelioma, mediastinal ganglioneuroblastoma, angiosarcoma and epithelioid sarcoma. CONCLUSION: Lymph node aspirates should be examined for alien cells, particularly smears that are paucicellular and demonstrate cystic change. Lymph node metastasis of STT is rare and influences staging, treatment and prognosis. Enlarged regional nodes should be examined with FNAC.