Innovative Surgical Planning in Resecting Soft Tissue Sarcoma of the Foot Using Augmented Reality With a Smartphone.

Augmented or hybrid reality is a display technology that combines the real world with the virtual world; it permits digital images of preoperative planning information to be combined with the surgeon's view of the real world. Augmented reality (AR) can increase the surgeon's intraoperative vision by providing virtual transparency of the real patient and has been applied to a wide spectrum of orthopedic procedures, such as tumor resection, fracture fixation, arthroscopy, and component's alignment in total joint arthroplasty. We present a case of a male patient who presented with pain in the medial aspect of his left foot after he underwent an incomplete mass excision elsewhere where it turned out to be synovial sarcoma. Because the mass was small, impalpable, and deeply positioned beneath both the plantar and the medial plantar aponeuroses, it was impossible to preoperatively decide a plan for resection. We opted to use the aid of AR in the form of an application using the camera of a smartphone. We were able to excise the tumor with negative surgical margins. On 12-month follow-up, the patient is in complete remission and has optimal mobility and functionality of his foot. In conclusion, AR holds great potential for use in the future of orthopedic surgical oncology. We emphasize using it via a handheld device that we found to be optimal for planning resection of the small and relatively fixed tumor. Based on our literature review, this is the first case describing the surgical planning in resecting an impalpable synovial sarcoma of the foot using AR technology.

Mismatch repair protein deficiency in triple-negative breast carcinomas.

BACKGROUND: Breast cancer, particularly triple-negative breast cancer (TNBC), poses a significant global health burden. Chemotherapy was the mainstay treatment for TNBC patients until immunotherapy was introduced. Studies indicate a noteworthy prevalence (0.2% to 18.6%) of mismatch repair protein (MMRP) deficiency in TNBC, with recent research highlighting the potential of immunotherapy for MMRP-deficient metastatic breast cancer. This study aims to identify MMRP deficiency in TNBC patients using immunohistochemistry. METHODS: A retrospective cohort study design was used and included TNBC patients treated between 2015 and 2021 at King Hussein Cancer Center. Immunohistochemistry was conducted to assess MMRP expression. RESULTS: Among 152 patients, 14 (9.2%) exhibited deficient MMR (dMMR). Loss of PMS2 expression was observed in 13 patients, 5 of whom showed loss of MLH1 expression. Loss of MSH6 and MSH2 expression was observed in one patient. The median follow-up duration was 44 (3-102) months. Despite the higher survival rate (80.8%, 5 years) of dMMR patients than of proficient MMR patients (62.3%), overall survival did not significantly differ between the two groups. CONCLUSION: Approximately 9% of TNBC patients exhibit dMMR. dMMR could be used to predict outcomes and identify patients with TNBC who may benefit from immunotherapy.

Diffuse Sarcoidosis Presenting as Metastatic Malignant Disease-Like Picture: A Case Report.

BACKGROUND: Sarcoidosis is a chronic inflammatory condition characterized by the formation of granulomas, which can impact various organs and tissues throughout the body. It frequently affects the lungs and mediastinal lymph nodes. The presence of multiple lytic lesions in the spine can be concerning, as it may indicate an undiagnosed or advanced metastatic malignancy. We present an unusual and interesting case of sarcoidosis in which the patient had multiple lytic lesions in the vertebrae, which resembled the presentation of metastatic malignant disease. CASE DESCRIPTION: A 46-year-old woman with no known medical history presented to our cancer center complaining of neck pain and bilateral shoulder pain. After conducting extensive investigations, an atypical manifestation of sarcoidosis was identified. The patient was administered corticosteroids, which effectively managed the disease and resulted in a substantial improvement in her symptoms. CONCLUSIONS: Before considering surgical intervention or radiotherapy for cases involving multiple lytic lesions in the spine with an unknown primary cause, it is essential to conduct a comprehensive diagnostic evaluation. This thorough work-up is necessary to establish a clear diagnosis. If the patient's neurological condition permits, a complete assessment can help prevent substantial morbidity. In certain cases, a patient's condition may involve an atypical manifestation of pathologies that are not related to metastatic diseases, suggesting that simpler treatment approaches might be adequate.

Mixed sex cord-stromal tumor (gynandroblastoma) with malignant morphology involving both ovaries: a case report.

Mixed sex cord-stromal tumors, which consist of poorly differentiated Sertoli cells and Leydig cells and juvenile granulosa cell tumor tissue, are extremely rare. Most of these tumors are unilateral and stage I at the time of diagnosis; nonetheless, according to the available relevant English-language literature, these tumors maintain a malignant potential. We herein report a case involving a 15-year-old girl diagnosed with a mixed sex cord-stromal tumor (gynandroblastoma with juvenile granulosa cell tumor component). Left salpingo-oophorectomy was initially performed, and the diagnosis of a juvenile granulosa cell tumor was established. Right salpingo-oophorectomy was performed 1 year later, at which time the specimen showed a different growth pattern involving epithelioid cells and tubules, resembling a Sertoli-Leydig cell tumor. Immunohistochemical staining was performed and the specimen was compared with that obtained 1 year earlier. We concluded that the tumors were linked and most likely constituted a gynandroblastoma (mixed form of sex cord-stromal tumor). Although this is an extremely uncommon ovarian tumor, it should be considered when diverse tumor morphology is identified. Bilateral metachronous involvement of the ovaries is possible. The grade of the Sertoli-Leydig cell component may influence the prognosis of such a tumor.

Flow cytometric signature of CD371-positive B-cell acute lymphoblastic leukemia.

OBJECTIVES: Multiparametric flow cytometry (MFC) aids in the diagnosis and management of B-cell acute lymphoblastic leukemia (B-ALL) by establishing a baseline immunophenotype for leukemic cells and measuring minimal residual disease (MRD) throughout the course of treatment. Aberrant expression patterns of myeloid markers in B-ALL are also examined during long-term surveillance. Here, we investigated the utility of the newly described myeloid marker cluster of differentiation (CD)371 in MRD surveillance via MFC in patients with CD371-positive B-ALL. METHODS: Eight-color MFC with standard panels (including CD371) was used to evaluate 238 patients with newly diagnosed B-ALL. Expression levels of key markers were retrospectively assessed at diagnosis, as well as days 15 and 33 of therapy. RESULTS: CD371 was expressed in 8.4% of patients with B-ALL. CD371 positivity was associated with older age at diagnosis, higher expression levels of CD34 and CD38, and lower expression levels of CD10 and CD20. Residual leukemic cells demonstrated decreased CD10 expression and increased CD45 expression after therapy, whereas CD371 expression remained stable. CONCLUSIONS: Patients with CD371-positive B-ALL exhibit a specific signature that merits further analysis, particularly because it has been associated with DUX4 rearrangement.

Transcervical approach to oropharyngeal synovial sarcoma: a case report.

Aim: Synovial sarcomas (SS) are malignant tumors rarely arising in the head and neck region. In most of these cases, the tumor arises in the cervical or hypopharyngeal region, and extremely rarely in the oropharynx. Case report: Herein, we report the case of a 22-year-old male oropharyngeal SS patient presented with breathing difficulty and dysphagia. The management plan included an emergency tracheostomy, followed shortly by transcervical resection of the oropharyngeal sarcoma tumor, the pectoralis major myocutaneous flap was used for pharyngeal reconstruction, followed by adjuvant radiotherapy resulting in more than 5 years disease-free survival. Conclusion: SS arising in the oropharynx are extremely rare. Transcervical resection coupled with adjuvant radiotherapy warrants enhanced locoregional control in advanced oropharyngeal cases.

Pembrolizumab for the Treatment of Relapsed and Refractory Classical Hodgkin Lymphoma After Autologous Transplant and in Transplant-Naïve Patients.

INTRODUCTION: Checkpoint inhibitors demonstrated significant efficacy in relapsed/refractory Hodgkin's Lymphoma (R/R cHL) resulting in high responses and prolonged progression free survival in patients, who relapse after or are ineligible for autologous stem cell transplantation (auto-SCT). We aimed to assess the efficacy and toxicity of Pembrolizumab before auto-SCT and in transplant naïve patients and calculate survival outcomes. PATIENTS AND METHODS: Fifty-five patients with R/R cHL were included. Patients demographics, including age, sex, risk stratification, therapy received and details pertaining transplantation, were collected. RESULTS: Median age was 28 years (range, 16-62 years). The median follow-up was 15.3 months and the median number of previous treatments was 3 (1-10). The best objective response was 74.5% (CR 32.7%, SD 5.5%) with reasonable safety profile. Twenty-nine of the responding patients received subsequent auto-SCT and 9 allogeneic stem cell transplantation (allo-SCT), 6 are currently alive with ongoing response. At the time of analysis, 6 patients remained on Pembrolizumab and the rest discontinued. The main reason for discontinuation was disease progression (n-49). Twelve-months overall survival and progression free survival (PFS) was 92% (95% CI: 76%-95%) and 51% (95% CI, 39%-67%) respectively. Twelve-month PFS for patients, who achieved CR or PR or PD was 88% (95% CI: 07%-75%); PR 60% (95% CI: 21%-29%) and 5% (95% CI: 5%-0%). Though the number of patients who received auto-SCT after Pembrolizumab was small (n-15), 12 months overall survival and PFS 100% and PFS 92%. 11 patients (20%) deceased during the follow-up and none was regarded to be treatment-related. CONCLUSION: Checkpoint inhibitors are effective in heavily pretreated cHL patients with reasonable survival outcomes. The results supporting the concept of auto and/or allo-SCT after checkpoint inhibitors use.

Metabolic and functional imaging employment in the differentiation of brown tumors from bone metastases in a case of primary papillary thyroid cancer and parathyroid adenoma: case report.

BACKGROUND: Brown tumors are benign osteoclastic bone lesions encountered in patients with hyperparathyroidism. These tumors may demonstrate aggressive, destructive features in the skeleton and imitate metastatic bone lesions, particularly in patients with known primary neoplasm. In this case report of recurrent papillary thyroid cancer and ectopic parathyroid adenoma, we shed light on the importance of combining different nuclear medicine imaging modalities to differentiate brown tumors from metastatic bone lesions. CASE PRESENTATION: We present a 39-year-old woman with a known history of papillary thyroid carcinoma classic type stage pT1N1b post-total thyroidectomy and radioactive iodine (I-131) therapy (RAI) presented with upper limb weakness and pain. An expansile lytic lesion involving the 6th cervical vertebra was seen in cervical spine MRI, which was suspicious for metastatic deposit. Therapeutic and diagnostic I-131 whole-body scans were negative for any I-13-avid lesions. Laboratory results revealed high calcium, parathyroid hormone, and alkaline phosphatase. A Technetium-99m-sestamibi (Tc-99m MIBI) scan was done with the standard protocol of spot views to the neck and upper chest area to localize any suspicious parathyroid adenoma. The scan demonstrated right supraclavicular and mediastinal Tc-99m MIBI-avid lesions suspicious for being ectopic parathyroid adenomas. Whole-body fluorine-18-2-fluoro-2-deoxy-D-glucose (18F-FDG), positron emission tomography/computed tomography (PET/CT) (18F-FDG PET/CT) was performed for further evaluation. It demonstrated multiple focal lytic skeletal lesions of abnormal increased FDG uptake as well as right supraclavicular FDG-avid lymph nodes. However, the superior mediastinal lesion was non-FDG-avid, suggesting the existence of two different entities: ectopic parathyroid adenoma with multiple brown tumors and metastatic right supraclavicular lymph nodes. The patient underwent right neck dissection and superior mediastinal mass excision. An intra-operative fresh serum parathyroid sample was sent, which dropped down to 100ng/ml from 863.7ng/ml. Later, histopathological results revealed that the right supraclavicular lymph nodes were metastatic papillary thyroid carcinoma. At the same time, the superior mediastinal mass proved to be parathyroid adenoma by histopathology, confirming the 18F-FDG PET/CT findings. CONCLUSIONS: In the case of papillary thyroid carcinoma, metastatic lymph nodes with hyperparathyroidism, and evidence of lytic bone lesions, careful interpretation of the different metabolic and functional imaging modalities are needed to exclude the concurrent parathyroid adenoma and facilitate the differentiation of brown tumors from bone metastases, leading to appropriate surgical and medical treatment plans.

Prolonged Neutropenia and Yellowish Discoloration of the Skin, But Not the Sclera, Following Excessive Turmeric Raw Root Ingestion.

The medicinal use of curcumin has gained popularity in recent years especially so among cancer patients undergoing chemotherapy. In this report, we describe the case of a 51-year-old female breast cancer patient who self-medicated on large amounts of turmeric root infusions while receiving chemotherapy. The patient presented with yellowish discoloration of her skin, but normal-colored sclera. She also had severe neutropenia, which persisted despite halting chemotherapy. When her white blood cell counts returned to normal, only after stopping her turmeric regimen, we determined that her neutropenia is associated with turmeric consumption making this the first report to establish this link. This report demonstrates that, as an alternative form of medication, curcumin consumption should still be monitored in cancer patients. We provide the visible sign of yellowish skin discoloration as a visible aid for healthcare providers in detecting turmeric consumption as a risk factor to be considered in differential diagnoses of unexplained neutropenia.

Concomitant Intraocular Retinoblastoma and Choroidal Hemangioma in a 1-Year-Old Boy.

We report a case of concomitant choroidal hemangioma in an eye that harbored intraocular retinoblastoma (RB) in a 1-year-old child. A 12-month-old boy presented with right white pupil (Leukocoria). The initial clinical diagnosis was unilateral intraocular RB Group C. The eye was treated initially by systemic chemotherapy combined with focal consolidation therapy followed by I-125 plaque radiotherapy. Massive sub-retinal recurrence was seen 4 months later, and Magnetic Resonance Imaging (MRI) showed extra-scleral invasion of the tumor, and therefore enucleation was performed. Microscopic examination revealed a differentiated retinoblastoma associated at the site of the base of the tumor with choroidal hemangioma with trans-scleral invasion. Orbit MRI was repeated 3 months after the surgery, and there was no orbital tumor recurrence. We are reporting a rare case of pathologically concomitant choroidal hemangioma with trans-scleral invasion in an eye that harbored active recurrent intraocular RB. This trans-scleral extension of hemangioma was misinterpreted by MRI as extraocular retinoblastoma and resulted in enucleation.

Characteristics, management, and outcome of squamous carcinoma of the conjunctiva in a single tertiary cancer center in Jordan.

AIM: To evaluate the features and outcome of management of malignant conjunctival squamous tumors in King Hussein Cancer Center (a referral tertiary cancer center in the Middle East). METHODS: Retrospective case series of 31 eyes for 31 patients with conjunctival squamous neoplasia. Main outcome measures included: age, gender, laterality, tumor location, pathological features, tumor stage, treatment modality, and outcome. RESULTS: Twenty (65%) patients were males and median age was 58y. Twenty-two (71%) eyes had the tumor in the nasal quadrant. Tumor invasion to nearby structures was seen in 19 (61%) eyes, including the cornea, fornix, eyelid, and orbit in 17 (55%), 1 (3%), 2 (6%), and 3 (10%) eyes, respectively. Eye salvage was achieved by surgical excision with cryotherapy followed by topical chemotherapy in 28 (90%) eyes, and orbital exenteration was necessary in 3 (10%) eyes due to orbital tumor invasion. Tumor recurrence was seen in 7 (23%) eyes, and the significant predictive factors for recurrence were tumor extension onto the nearby structures (P=0.04), tumor invasiveness (P=0.038), and tumor TNM stage (P=0.031). No significant change in visual acuity was seen, and disease related mortality was 6% (2 patients, both had orbital invasion by invasive squamous carcinoma). CONCLUSION: Conjunctival squamous carcinoma is more common in males. Advanced American Joint Committee on Cancer (AJCC) T-stage, tumor local invasion, more pathologically aggressive tumors, and surgical treatment alone (without adjuvant therapy) are associated with higher risk for recurrence, and orbital invasion is the most important poor prognostic factor for metastasis and death. Treatment strategies should be affected by tumor characteristics at presentation.

Reliability of frozen section in breast sentinel lymph node examination.

OBJECTIVES: Sentinel lymph node (SLN) examination in breast carcinoma is crucial to spare patients unnecessary lymph node (LN) dissection. The specificity and accuracy of SLN examination by frozen section has been variable in many studies. This study aims to describe our experience in frozen section (FS) analysis of SLN. METHODS: We have retrospectively analyzed data from 449 axillary SLN subjected to FS examination from 440 patients with breast cancer. All patients had free axillary LN clinically. RESULTS: Out of 449 cases, no false positive cases were found on FS (specificity of 100 %). Twenty-six cases were false negative (6 interpretation-related reasons and 20 technical-related reasons). The overall sensitivity was 84 % with a total accuracy rate of 93 % and interpretation sensitivity of 96 %. Three cases were deferred. Twenty-two of the false negative cases were micrometastases, whereas 4 were macrometastases. The interpretation-related false negative cases were not related to the subtype of carcinoma (ductal vs. lobular). However, they were all of low nuclear grade. CONCLUSION: These findings are similar to most published data. FS is a reliable method for evaluating SLN. The most common cause of false negative diagnosis is sampling error. More attention should be paid to low-grade tumors. Moreover, in FS analysis, we recommend to totally submit SLNs that are less than 5 mm in diameter, bisecting them if possible, and to serially section SLNs that are at least 5 mm at 2-mm intervals.

Residual monotypic plasma cells in patients with waldenstrom macroglobulinemia after therapy.

Waldenström macroglobulinemia (WM) is currently defined as lymphoplasmacytic lymphoma involving bone marrow (BM) associated with a serum IgM paraprotein. WM is typically composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells in variable proportions, which can change after therapy. In this study, we assessed 41 WM cases that required chemotherapy, 39 showing persistent disease in restaging BM specimens. In 10 cases, there was persistent monotypic plasmacytosis in BM in the absence of demonstrable monotypic B cells. The monotypic plasma cells represented 0.5% to 46% of the cellularity and persisted 1 to 50 months after the last course of chemotherapy. The plasma cells were best quantified by immunohistochemical analysis on paraffin sections. We conclude that WM can persist as a pure plasma cell population after therapy. This finding has implications for the immunophenotypic assessment of WM after therapy and may explain persistent IgM paraproteinemia in patients with WM with no evidence of a clonal B-lymphocyte population.

Detection of nucleophosmin 1 mutations by quantitative real-time polymerase chain reaction versus capillary electrophoresis: a comparative study.

CONTEXT: Nucleophosmin 1 (NPM1) is the most commonly mutated gene in acute myeloid leukemia. Detection of NPM1 mutations is useful for stratifying patients for therapy, predicting prognosis, and assessing for minimal residual disease. Several methods have been developed to rapidly detect NPM1 mutations in genomic DNA and/or messenger RNA specimens. OBJECTIVE: To directly compare a quantitative real-time polymerase chain reaction (qPCR) assay with a widely used capillary electrophoresis assay for detecting NPM1 mutations. DESIGN: We adopted and modified a qPCR assay designed to detect the 6 most common NPM1 mutations and performed the assay in parallel with capillary electrophoresis assay in 207 bone marrow aspirate or peripheral blood samples from patients with a range of hematolymphoid neoplasms. RESULTS: The qPCR assay demonstrated a higher analytical sensitivity than the capillary electrophoresis 1/1000 versus 1/40, respectively. The capillary electrophoresis assay generated 10 equivocal results that needed to be repeated, whereas the qPCR assay generated only 1 equivocal result. After test conditions were optimized, the qPCR and capillary electrophoresis methods produced 100% concordant results, 85 positive and 122 negative. CONCLUSIONS: Given the higher analytical sensitivity and specificity of the qPCR assay, that assay is less likely to generate equivocal results than the capillary electrophoresis assay. Moreover, the qPCR assay is quantitative, faster, cheaper, less prone to contamination, and well suited for monitoring minimal residual disease.

Characteristics of gastrointestinal stromal tumors in a Middle Eastern population.

OBJECTIVE: To demonstrate the immunohistochemical and epidemiological characteristics of gastrointestinal stromal tumors (GIST) in a Middle Eastern population. METHODS: This is a retrospective analysis of all intra-abdominal mesenchymal tumors (excluding childhood embryonal rhabdomyosarcoma and small round blue cell tumors) collected from the archives of the Pathology Departments of King Hussein Cancer Center, Amman, and King Abdullah University Hospital, Irbid, Jordan between 2001 and 2008. The immunohistochemical profile of all cases was studied at King Hussein Cancer Center, Amman, Jordan, between January and August 2009. RESULTS: Gastrointestinal stromal tumors comprised 45% of the intra-abdominal mesenchymal tumors (42 out of 93 cases), with the most common site being the stomach (n=17, 40.5%). Twenty-seven GIST cases (64.3%) were classified as high risk, 4 (9.5%) as intermediate risk, 6 (14.3%) as low risk, and 2 (4.8%) as very low risk. Immunohistochemistry showed diffuse and strong positivity (+3) for CD117 in 85.7% of GIST cases, and for CD34 in 65% of cases. The high-risk tumors were more common in male patients (M:F=1.7:1), while the non-high risk tumors were more common in female patients. CONCLUSION: The immunohistochemical profile of GIST in Jordanian patients is similar to previously published data from other populations, with a slight male preponderance for high-risk GISTs.