Primary Clear Cell Sarcoma of the Lung: a Case Report.

BACKGROUND: Clear Cell Sarcoma of Soft Tissue (CCSST), or melanoma of the soft part, is a rare, aggressive tumor that originates in the aponeurosis and fasciae of the distal parts of the extremities. Reports from other sites of the body are rare. OBJECTIVE: We are reporting an extremely rare tumor that presented as a central left-sided lung mass and found to be clear cell sarcoma of soft tissue. METHODS: We report a 24-year-old male patient presented with recurrent attacks of left-sided chest pain associated with cough and dyspnea. RESULTS: Imaging showed a central left-sided 8*5.5*5 cm lung mass. The age of the patient and the radiological characteristics of the lesion were suggestive of a benign pathology. After histopathological assessment of the lesion, suspicion of the malignant process was raised, mainly melanoma of soft part and PEComa. The patient underwent left-sided pneumonectomy. The postoperative histological examination, immunohistochemical findings including positive staining for S-100, HMB-45, and Melan-A, and positive FISH study for EWSR1 gene rearrangements supported the diagnosis of CCSST originating primarily in the major fissure of left the lung. CONCLUSION: The rarity of CCSST in general and tumors originating in the lung primarily raise the challenges in hypothesizing a differential diagnosis, choosing proper investigations and treatment methods. The histological examination, immunohistochemical, and cytogenetics of the tumor are mandatory to reach the final diagnosis.

Retrospective implementation of the Milan system for reporting salivary gland cytopathology; a review of 5 years in a specialized cancer center.

BACKGROUND: The Milan system for reporting salivary gland cytopathology (MSRSGC) aims to standardize terminology, facilitate communication, and optimize management by providing risk of malignancy (ROM) for each category. Our retrospective cohort aims to study the reproducibility of reporting using the MSRSGC and to calculate the ROM for each category. METHODS: Cases of fine needle aspiration (FNA) of salivary glands and related cervical lymph nodes were retrieved from our files between 2015 to 2019. From a total of 63 cytology cases, 57 cases had available material for cytological reexamination of which 45 cases had follow up data. All cases were reviewed independently by two pathologists and reclassified based on the MSRSGC. The reclassification of cases for both pathologists was compared and the ROM for each diagnostic category was calculated. RESULTS: The 57 cases were studied. Both pathologists had initial concordance in classification of 52 of 57 cases. The remainder five cases were concurred upon after combined review. The cases were classified as: Non Diagnostic (ND); (n = 8), Non Neoplastic (NN); (n = 7), Atypia of Undetermined Significance (AUS); (n = 8), Neoplasm Benign (NB) (n = 10), Salivary Gland Neoplasm of Uncertain Malignant Potential (SUMP) (n = 5), Suspicious for Malignancy (SM) (n = 1) and Malignant (M) (n = 18). The ROM was: ND: (33.3%); NN: (0%); AUS (33.3%); NB (0%); SUMP (25%); SM (100%) and M (100%). CONCLUSION: Applying the MSRSGC is reproducible which facilitates standardization of reports and stratifying cases preoperatively. In general, the ROM for our cases was close to that reported in the literature.

Novel Double Central Ray Amputation of the Third and Fourth Digits: Case Report and Literature Review.

Whenever partial hand amputations for soft tissue sarcomas are attempted, special consideration should be given to achieve a balance between complete resection associated with negative margins and preservation of functionality to the patient so that the hand can support the contralateral intact hand for bimanual activities. This difficult decision is even more challenging within the limited anatomical confines of the hand. Based on our literature review, this is the first case of double central 3rd and 4th ray amputation, as far as we know with good hand function, evaluated by the Musculoskeletal Tumor Rating Scale.

Metastatic poorly differentiated chordoma: the eyes do not see what the mind does not know

Chordoma is a rare tumor. It has unique clinical, pathological and immunohistochemical characteristics. Accurate diagnosis is essential as the tumor shows an aggressive clinical course and requires a multimodal therapeutic approach. A case with wide spread distant metastatic disease that was initially thought to represent metastatic thyroid carcinoma is presented. Appropriate clincopathologic correlation and the histologic findings raised the possibility of poorly differentiated chordoma. The diagnosis was confirmed by immunohistochemistry for INI-1 and Brachyury. The approach to the diagnosis emphasizing the clinical and pathologic findings of this case is discussed and reviewed in the context of the published literature.

Metastatic poorly differentiated chordoma: the eyes do not see what the mind does not know.

Chordoma is a rare tumor. It has unique clinical, pathological and immunohistochemical characteristics. Accurate diagnosis is essential as the tumor shows an aggressive clinical course and requires a multimodal therapeutic approach. A case with wide spread distant metastatic disease that was initially thought to represent metastatic thyroid carcinoma is presented. Appropriate clincopathologic correlation and the histologic findings raised the possibility of poorly differentiated chordoma. The diagnosis was confirmed by immunohistochemistry for INI-1 and Brachyury. The approach to the diagnosis emphasizing the clinical and pathologic findings of this case is discussed and reviewed in the context of the published literature.

TAZ and YAP are frequently activated oncoproteins in sarcomas.

TAZ (WWTR1) and YAP are transcriptional coactivators and oncoproteins inhibited by the Hippo pathway. Herein we evaluate 159 sarcomas representing the most prevalent sarcoma types by immunohistochemistry for expression and activation (nuclear localization) of TAZ and YAP. We show that 50% of sarcomas demonstrate activation of YAP while 66% of sarcomas demonstrate activated TAZ. Differential activation of TAZ and YAP are identified in various sarcoma types. At an RNA level, expression of WWTR1 or YAP1 predicts overall survival in undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma. Immunohistochemistry demonstrates that TAZ and YAP expression and activation are positively correlated with grade in the well-differentiated liposarcoma to dedifferentiated liposarcoma tumor progression sequence as well as conventional chondrosarcomas. TAZ and YAP are constitutively activated oncoproteins in sarcoma cell lines. Knock-down of TAZ and YAP demonstrate differential activity for the two proteins. Verteporfin decreases colony formation in soft agar as well as CTGF expression in sarcoma cell lines harboring activated TAZ and YAP.

Alveolar Soft Part Sarcoma.

Alveolar soft part sarcoma is a rare neoplasm usually arising in the soft tissues of the lower limbs in adults and in the head and neck region in children. It presents primarily as a slowly growing mass or as metastatic disease. It is characterized by a specific chromosomal alteration, der(17)t(X:17)(p11:q25), resulting in fusion of the transcription factor E3 (TFE3) with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25. This translocation is diagnostically useful because the tumor nuclei are positive for TFE3 by immunohistochemistry. Real-time polymerase chain reaction to detect the ASPSCR1-TFE3 fusion transcript on paraffin-embedded tissue blocks has been shown to be more sensitive and specific than detection of TFE3 by immunohistochemical stain. Cathepsin K is a relatively recent immunohistochemical stain that can aid in the diagnosis. The recent discovery of the role of the ASPSCR1-TFE3 fusion protein in the MET proto-oncogene signaling pathway promoting angiogenesis and cell proliferation offers a promising targeted molecular therapy.

Is Hashimoto's thyroiditis a risk factor for medullary thyroid carcinoma? Our experience and a literature review.

The etiology of medullary thyroid carcinoma remains unknown. The aim of this study was to determine whether there is a significant association between medullary thyroid carcinoma and Hashimoto's thyroiditis in the histopathologic material of thyroidectomized patients. Retrospective cross-sectional study. In this study, we reviewed the medical records of all patients who underwent total thyroidectomy for different thyroid-related complaints between January 2000 and January 2012 at Jordan University Hospital-Amman, Jordan. To highlight relevant previously published studies addressing this topic, a literature search was conducted for English language studies reporting "medullary thyroid carcinoma" or "C-cell hyperplasia" in patients with Hashimoto's thyroiditis. Of the 863 patients with a mean age of 47.2 ± 12.3 years who underwent total thyroidectomy during the study period, 78 (9.04 %) were diagnosed with Hashimoto's thyroiditis, and 15 (1.74 %) had medullary thyroid carcinoma, 3 (20 %) of whom had coexistent Hashimoto's thyroiditis. A total of 683 (79.1 %) patients had benign thyroid disease, 67 (9.8 %) of whom had Hashimoto's thyroiditis. The difference between these rates was not statistically significant (p = 0.19). When examined by gender, 9 females had medullary thyroid carcinoma, 3 (33.3 %) of whom had coexistent Hashimoto's thyroiditis; by contrast, of 560 females with benign thyroid disease, 62 (11.1 %) had Hashimoto's thyroiditis (p = 0.04). Although this study population represents a small and single-institution experience, our results suggest that there might be an association between Hashimoto's thyroiditis and medullary thyroid carcinoma only in female patients who undergo total thyroidectomy.

Spinal cord schistosomiasis: unexpected postmortem finding.

OBJECTIVES: To report a case of a US resident, originally from Liberia, with chronic hepatitis C infection who developed acute neurologic symptoms of the lower limbs. METHODS: Our case is compared to previously reported similar cases, with emphasis on clinical symptoms, investigations, diagnosis, and prognosis. RESULTS: The patient was transferred to the University of Iowa Hospital and Clinics for further management of severe retroperitoneal bleeding and died 2 days after admission. The diagnosis of schistosomiasis was established on examination of the spinal cord at autopsy, where multiple Schistosoma mansoni eggs were seen in the vasculature of the spinal cord. CONCLUSIONS: The diagnosis of schistosomiasis may go undiagnosed in countries where the disease is not endemic but should be considered when investigating spinal cord disease in patients native to an endemic area or international travelers.