Case Reports in Pathology最新文献

Solitary fibrous tumors are rare tumors of mesenchymal origin. Although most often observed in the lung pleura, they have been reported in varied extrapleural sites. A 70-year-old male with complicated Crohn's disease presented with 3 days of nausea, emesis, constipation, and abdominal pain. Computed Tomography (CT) demonstrated mucosal thickening of the middescending colon, consistent with fibrosing stricture. Surgical excision revealed an unusual, 5 cm mass originating in the subserosa. Histopathology of the lesion was notable for a proliferation of cells with spindle and stellate-shaped nuclei and no appreciable mitotic figures, which extended into the muscularis and submucosa. Immunohistochemistry was STAT6 nuclear positive and cytoplasmic CD34 positive, diagnostic for solitary fibrous tumor (SFT). In this case, the SFT infiltrating into the muscularis propria and subserosa caused the stricture and bowel obstruction. This illustrates that while fibrosing strictures are usually the etiology of bowel obstruction in the setting of Crohn's disease, other rare possible causes should be considered.

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with widespread distribution throughout the body and unpredictable clinical behavior. Recently, a subset of these tumors has been reported to harbor Transcription Factor E3 (TFE3) gene rearrangement with distinct morphologic and immunophenotypic features. Although limited, these tumors may represent a separate entity from the conventional PEComas and may require different treatment approaches. Surgery is the main treatment option with no clear consensus on systemic therapy. Here, we present the first case of a malignant pediatric colonic TFE3-associated PEComa with isolated liver metastasis leading to liver transplantation for the local control.

Carcinosarcomas (CSs) of the endometrium have admixture of malignant epithelial and mesenchymal components. The carcinomatous component exhibit endometrioid, serous, or clear cell differentiation, or are undifferentiated. CSs are considered homologous or heterologous according to the type of sarcomatous component. Sertoliform endometrioid carcinomas (SECs) of the endometrium which comprise a rare subtype of endometrial cancer, typically occur in the ovary. SECs as a carcinomatous component of CS of the endometrium have not been reported. Here, we report an endometrial carcinosarcoma that contains an SEC component. An 88-year-old female presented to a clinic with atypical genital bleeding. She was referred to our hospital and underwent total hysterectomy, bilateral adnexectomy and partial omentectomy due to endometrial carcinoma. Gross examination revealed a polypoid mass in the uterine cavity with massive myometrial invasion. Histologically, the tumor was a high-grade endometrioid carcinoma. In addition to an ordinary conventional endometrioid carcinoma, approximately 30% of the area exhibited sex cord-like pattern and contained small hollow tubules, anastomosing cords and trabeculae, and tightly packed nests. Immunohistochemically, the SEC component showed diffuse p53 staining. Sex cord-like area, especially the solid area, showed positive staining for EMA, vimentin, α-inhibin, CD99, calretinin, p53, CD56, synaptophysin, and chromogranin A, which is a staining pattern similar to that previously reported SEC of the endometrium. Diminished membranous and positive cytoplasmic staining for β-catenin was observed. This is the first case report of an endometrial carcinosarcoma containing an SEC component. SECs of the endometrium might exhibit sex cord-like differentiation in contrast to SECs of the ovary, which do not exhibit sex cord differentiation.

Progressive nodular histiocytosis (PNH) is a rare condition characterized by progressive eruption of multiple yellowish-brown papules and nodules on the skin and mucous membranes. We present the case of a 37-year-old Caucasian man with gradually increased appearance of nodular lesions on the forehead and right temple. These lesions were initially diagnosed as xanthomas and did not respond to intralesional injections of triamcinolone. Additional biopsy revealed an intense dermal infiltrate of foamy mononuclear epithelioid cells with a minor admixture of plasma cells, lymphocytes, and scattered multinucleated giant cells. On immunohistochemical staining, the lesional cells were positive for CD163 and CD68 and negative for CD1a, thus confirming a mononuclear-macrophage lineage. The clinical presentation and the histological impression lead to the diagnosis of PNH. This condition could be challenging, mimicking microscopically similar lesions of the non-Langerhans cell histiocytosis group. Although uncommon, PNH stands out due to its clinical and microscopic features and should be taken into consideration in the differential diagnosis of cutaneous histiocytoses.

Introduction: Gastrointestinal angiosarcomas are rare and represent less than 1% of all gastrointestinal tract malignancies, with most occurring in the stomach and small intestine. Occurrence in the colorectal segments is considered extremely rare. Case Report. We describe the case of a 61-year-old male with multiple primary angiosarcomas of the colon who presented with fever and abdominal pain. The patient was initially hospitalized and treated as having an infectious disease. A multislice computed tomography (MSCT) scan revealed multiple soft tissue tumors in the region of the left iliopsoas and gluteus medius muscles. After developing hematochezia, a colonoscopy was performed which found an ulcerated tumor in the sigmoid colon. The small tissue biopsy taken during the procedure presented diagnostic difficulties and was given a preliminary diagnosis of gastrointestinal stromal tumor (GIST). Examination of the resected colon segment and surrounding fat tissue revealed four separate tumors. Microscopically, the tumors were composed of solid sheets of spindle and epithelioid neoplastic cells with prominent nucleoli and numerous mitotic figures and immunohistochemically positive for ERG, CD31, CD34, vimentin, and CD117, while negative for CK7, CK20, CD20, CD3, CD45, TTF-1, PAN-CK, ALK, Mpox, S-100, and DOG1, leading to the final diagnosis of multiple colonic angiosarcomas. The patient's condition declined rapidly and he passed away from multiple organ failures 60 days after initial hospitalization.

Conclusion: Both clinical and pathological diagnoses of colorectal angiosarcoma are challenging. Patients are present with nonspecific symptoms leading to mismanagement and late diagnosis. A definitive pathological diagnosis relies on immunohistochemical staining for endothelial markers. Misdiagnosis as poorly differentiated adenocarcinoma or GIST is possible in limited tissue biopsies.

Primary dural lymphoma (PDL) usually arises from the calvarial dura without the brain parenchyma or systemic involvement and thus may not be considered as a typical form of primary CNS lymphoma (PCNSL). It is exceedingly rare. When it occurs, it might not be suspected as a primary diagnosis on clinical and radiologic findings. We present a PDL case that occurs at the cerebellopontine (CP) angle mimicking en plaque meningioma. The tumor histopathology showed a lymphoproliferative disorder immunophenotypically consistent with a low-grade marginal zone lymphoma. Bone marrow and systemic involvements were not identified, and a diagnosis of PDL was established. As a residual tumor at the CP angle was inaccessible to surgery, postoperative radiation therapy was performed. No recurrence was found at 15-month follow-up. PDLs are mostly indolent and have a good prognosis. There is no doubt that the most important differential diagnosis is meningioma. Furthermore, the present case emphasizes the necessity of an intraoperative consultation and knowledge of this rare yet essential form of PCNSL so that appropriate studies can be ordered.

Retroperitoneal metastasis of seminoma often occurs in the higher stage through lymph nodes. Generally, seminoma expresses specific germ cell markers while being negative for carcinoma markers. We present a unique case of cytokeratin positive seminoma initially presented as retroperitoneal metastasis. The diagnosis was made based on the histological features and immunohistochemical stains. Testicular ultrasound confirmed the primary tumor in the patient's left testicle. Pathologists should always be aware of germ cell tumors when encountering a metastasis of an unknown primary.

[This corrects the article DOI: 10.1155/2019/2732674.].

Solitary fibrous tumors (SFTs) are rare mesenchymal tumors, originally identified in the pleura. Even though they have subsequently been described in several extrapleural sites, the incidence of SFTs in the spermatic cord is particularly rare. Here, we report a case of a 27-year-old male that presented with a 3-year history of left scrotal swelling. Computed tomography (CT) and ultrasound demonstrated multiple solid, hypoechoic well-circumscribed masses that were separate from the testis. Surgical excision of the mass led to pathologic diagnosis of a solitary fibrous tumor involving the spermatic cord. Solitary fibrous tumors, although rare, are an important differential diagnosis for urogenital tumors.

Primary leiomyosarcomas (LMS) of vascular origin are rare tumors, and more than half of the cases arise in the inferior vena cava (IVC). Primary LMS of the renal vein are extremely rare tumors with only a few cases reported in the literature. Their diagnosis is made only by pathological features. Histologically, they are made of atypical spindle-shaped cells arranged in long intersecting fascicles. Tumor cells stain positive for myogenic markers in immunohistochemistry. Standard treatment consists of radical nephrectomy followed by chemotherapy and/or radiotherapy. Because of insufficient histological data and follow-up, the prognosis factors are not well identified. Overall prognosis of renal vein LMS is poor. We report here an exceptional case of a huge LMS of the right renal vein mimicking a primitive renal cell carcinoma, occurring in a 56-year-old male patient.