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Sclerotic fibroma (SF) is a rare subset of dermal fibromas that occurs sporadically or in association with Cowden syndrome (CS). We report a case of a patient with known CS and a solitary lesion on the scalp. Histologic examination demonstrated a well-circumscribed lesion with sclerotic dermis and a whorled collagen pattern, multinucleated giant cells, and dendritic spindle cells. Nuclear atypia or mitotic figures were not noted. The giant cells were negative for Melan-A, SOX-10, EMA, SOX-10, and factor XIIIa. These findings are consistent with a giant cell collagenoma (GCC). Despite possible overlap with SF, GCC has not been associated with CS. This makes our case unique and suggests that GCC should be included in the spectrum of CS-associated cutaneous lesions. The diagnosis of SF may lead to the identification of previously undiagnosed CS; accordingly, GCC, even when present as a solitary lesion, may indicate the need for further work-up and screening for CS.

The therapeutic approach of pleomorphic liposarcoma (PLPS), a rare high-grade subgroup of soft tissue sarcoma, is commonly extrapolated from the management of other LPS subtypes. Only published retrospective data on PLPS currently serve as a guide for oncologists without clear recommendations or specific guidelines. In the advanced setting, specific systemic therapy such as eribulin and trabectedin showed promising activity in comparison to conventional therapy (doxorubicin- and gemcitabine-based protocols), which currently remains the current standard of care at initial stages of the disease. The better understanding of soft tissue sarcoma (STS) pathophysiology and disease course has led to the development of adapted clinical trial designs for rare STS histotypes with specific treatment approach.

UNASSIGNED: Primary hepatic sarcomas are one of the extremely rare malignant tumors representing less than 0.1 % of all primary malignant neoplasms.
UNASSIGNED: A known compensated cirrhotic HCV 60-year-old female lady MELD score 8 with a medical history of controlled diabetes and hypertension presented to our hepatobiliary clinic experiencing a chronic right hypochondrial pain that radiate to her right shoulder of 3 months duration. O/E there was right hypochondrial and epigastric tenderness. Pelvi-abdominal US was done then PET CT which revealed a large exophytic focal lesion measuring about 8 × 7 × 6 cm and achieving 12.4 SUV max on FDG uptake. Pre-operative US guided true-cut biopsy showed small oval rounded cells with focal spindling and the neoplastic cells showed hyperchromatic pleomorphic nuclei with little cytoplasm with a positive reaction of tumor cells stained with Desmin and Myogenin with a diagnosis of pleomorphic rhabdomyosarcoma. The patient was scheduled for neoadjuvant chemotherapy and then elective Right hemihepatectomy. Although many challenges were encountered during the resection of the tumor as the tumor was attached to the under surface of the diaphragm, the tumor was resected with a safety margin of 1 cm with frozen histopathological examination being negative for any malignant cells. The patient\'s postoperative course was uneventful apart from wound infection and was discharged on the postoperative sixth day.
CONCLUSIONS: The percutaneous liver biopsy plays an important role in the diagnosis of liver\'s rhabdomyosarcomas. Neoadjuvant chemotherapy addresses the behavior of the tumor, together with early surgical intervention can lead to favorable outcomes and reduce the recurrence.

OBJECTIVE: Carcinoma ex Pleomorphic Adenoma (CXPA) is a rare primary salivary gland malignancy, typically arising from a pre-existing pleomorphic adenoma. This systematic review examines prognostic factors affecting overall survival (OS) in major and minor salivary gland CXPA.
METHODS: Systematic review of MEDLINE, Cochrane, Scopus, Web of Science, CINAHL, and Open Grey databases from inception to 31st March 2022 for all English-language literature pertaining to \'carcinoma ex pleomorphic adenoma\'. All study types with greater than five patients with CXPA of the major and minor salivary glands were eligible for inclusion.
RESULTS: Of 8143 studies, 39 studies (n = 5637 patients) meeting the inclusion criteria were included. Median OS at one, three, five, and ten years were 90.0 %, 72.0 %, 61.9 %, and 45.0 % respectively for all CXPA. Higher staging, T stage, nodal disease, grading, and invasion ≥ 1.5 mm had worse outcomes. Histological subtype, perineural invasion, and radiotherapy did not demonstrate a consistent trend. Three studies were evaluated to have high risk of bias, and was removed for sensitivity analysis.
CONCLUSIONS: Survival outcomes worsen with time for all salivary gland CXPA. Further research on histopathological features and the utility of radiation therapy is required to guide patient selection for more aggressive treatment.
BACKGROUND: CRD42021238544 (PROSPERO).

Sarcomatoid carcinoma is a subtype of non-small cell lung cancer (NSCLC) characterized by mesenchymal - epithelial transition component and awful prognosis. In this report, based on a case of stage IV lung sarcomatoid carcinoma with an extraordinary evolution and survival over 4 years, we address unresolved questions about the treatment of this cancer. We also make a literature review about the key factors that characterize this histology and that should be considered when treating those patients. Sarcomatoid carcinoma presents with mutations as KRAS, EGFR, ALK or MET in up to 70% of cases, and an important expression of PD-L1 (also called B7-H1), which can influence treatment of those patients with new drugs as immune checkpoint inhibitors. Immunotherapy has changed the horizon of patients with stage IV lung cancers without driver mutations, as their survival has improved extraordinary. Moreover, radical treatments are being considered in long survivors with oligometastatic disease. In this report, we review targeted and radical therapy, treatment duration and the mechanisms responsible of disease evolution of sarcomatoid tumors.

Solid pseudopapillary neoplasm of the pancreas (SPN) is a rare low-grade malignancy typically occurring in young women. Occasionally, these neoplasms present with pleomorphic to atypical multinucleated giant tumor cells which may mimic high-grade malignancy. Our patient is a 25-year-old male who presented with one year of intermittent epigastric pain. Magnetic resonance imaging showed a 3.1 × 2.5 cm mass in the pancreas body. Endoscopic ultrasound-guided fine needle aspiration of the mass showed large pleomorphic cells and atypical multinucleated giant cells in a background of singly scattered polygonal cells. Focally, these cells surrounded delicate hyalinized to fibrovascular cores forming pseudopapillae. Immunohistochemical stains show tumor cells are positive for beta-catenin, CD10, vimentin, and CD56. Although rare surgical pathology publications have described the presence of pleomorphic to atypical multinucleated giant cells occurring in SPN, to our knowledge, this is the first case reported example focused on cytomorphologic illustration and description.

Primary pleomorphic xanthoastrocytoma (PXA) of the spinal cord is a rare slow growing tumor. To our knowledge, only five such cases have been reported in the literature till date. We report the clinical, radiological, and histopathological features of a spinal PXA in a 23-year-old female previously operated 5 years back for a spinal tumor, presented with weakness in lower limbs, sphincter incontinence and low back pain. Magnetic resonance imaging scan with contrast reveals an intramedullary lesion in the spinal cord from D8-D10 level. The patient was operated with reexploration of the previous incision, and gross total excision was achieved. Histopathology confirmed the diagnosis of PXA. Clinical and radiological follow-up is required to detect early recurrence. Adjunct radiotherapy or and chemotherapy should be considered only when there is postoperative residual or recurrence, however there are no definite guidelines in view of the rarity of this condition.

Cervical high-grade squamous intraepithelial lesions (HSILs) are typically characterised by a proliferation of immature basaloid cells with relatively uniform hyperchromatic nuclei. In this report we describe 19 cases of HSIL exhibiting focal but very marked nuclear atypia often associated with multinucleation (\'pleomorphic HSIL\'). The bizarre cytological changes mainly involved the basal epithelium particularly in endocervical crypts where the neoplastic cells undermined the native glandular epithelial cells. Superficially invasive squamous cell carcinoma (SISCCA) was present in three cases (16%) and while this was more common than in a comparative series of 40 \'conventional\' HSIL excision specimens (5%), the difference was not statistically significant. All three invasive cases demonstrated additional histological features that have been associated with increased risk of SISCAA (expansile crypt involvement by HSIL, luminal necrosis, and/or intraepithelial squamous maturation), and the invasive foci were associated microanatomically with conventional-type rather than pleomorphic HSIL. The bizarre cells expressed p16 and p63 proteins but usually lacked mitotic activity and showed less Ki-67 labelling than adjacent conventional HSIL. These findings suggest that pleomorphic epithelial changes in HSIL do not necessarily indicate more aggressive biological behaviour and may, in some cases, represent a degenerative phenomenon.

Sarcomas account for less than 1% of all primary breast malignancies, pleomorphic sarcoma of the breast being even rarer. We present two cases of pleomorphic sarcoma of the breast in a 35-year-old and a 43-year-old female. An extensive review of the available literature with evaluation of the etiology, changing terminologies and histopathologic features of pleomorphic sarcoma of the breast are discussed. The prognostic factors and treatment modalities have also been reviewed.