Large gangliocytic paraganglioma of the duodenum: A rare entity

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.     

原发性腹膜后副神经节瘤14例报告并文献复习

目的：探讨原发性腹膜后副神经节瘤的临床和病理特点、诊治方法及预后。方法：回顾性分析14例病理检查证实为腹膜后副神经节瘤患者的临床资料：男8例,女6例,年龄14～66岁。有高血压症状9例,其中尿香草扁桃酸（VMA）升高8例,尿儿茶酚胺升高9例。14例均接受手术治疗。术后随访13个月～5年。结果：术中见肿瘤位于肾门区4例,肾上极区5例,肾下极区2例,肾上腺前外上方3例。13例手术完全切除肿瘤,1例伴肝转移者行肿瘤大部切除及肝尾叶切除。病理检查诊断为良性肿瘤13例,恶性肿瘤1例。术后随访12例,9例术前高血压者有7例血压恢复正常;9例无瘤生存,1例复发,2例死亡。结论：手术完全切除肿瘤是腹膜后副神经节瘤最有效的治疗方法,充分的术前准备和围手术期处理是手术成功的关键。术后生存时间取决于肿瘤是否发生淋巴结或远处转移,复发病例可再次手术切除。     

Mesenteric Paraganglioma: Report of a Case

We report a rare case of a solitary primary paraganglioma arising in the mesentery, found in a 72-year-old woman who presented with abdominal pain and a palpable abdominal mass. This extra-abdominal paraganglioma developed from paraganglionic cells that travelled by vertebral migration from the root of the superior mesenteric artery. Extra-adrenal paraganglia extend anywhere from the neck down to the base of the pelvis. Ultrasonography, computed tomography (CT), and angiography showed a solid and cystic heterogeneously enhanced mass, which was fed by the superior mesenteric artery, without distant metastasis. Exploratory laparotomy revealed a large, dark, brownish-red mass in the mesentery of the ileum, which was distinct from the ileum. The mass consisted of peripherally solid areas with central hemorrhage and cystic degeneration. It was diagnosed as a paraganglioma histologically. The patient is free from recurrence of paraganglioma after 1 year of follow up. To our knowledge, this represents only the seventh case of a paraganglioma arising in the mesentery.     

Nonfunctional Paraganglioma of the Pancreas: Report of a Case

(Received for publication on Apr. 30, 1996; accepted on Mar. 4, 1997)     

Spindle Cell Lipoma Arising from the Supraglottis: A Case Report and Review of the Literature

Lipomas are common benign mesenchymal neoplasms. Although 13% of lipomas are found in the head and neck, only 0.6% have been reported in the larynx. Of all lipomas, the spindle cell variant is the least common. In the present study, we report a case of supraglottic spindle cell lipoma and review the literature of laryngeal spindle cell lipoma. A 35-year-old male presented with dysphagia and dyspnea and was found to have bilateral supraglottic lesions causing airway obstruction. The masses were resected endoscopically. Final pathology demonstrated mature adipocytes and spindle cells, with immunohistochemical patterns supportive of spindle cell lipoma. Spindle cell lipomas have rarely been reported in the upper airway. To our knowledge, this is the youngest patient reported to date. These lipomas are uncommon benign neoplasms and should be distinguished from aggressive mesenchymal neoplasms such as liposarcoma variants to guide appropriate conservative but curative therapy.     

Epithelioid Hemangioma of the Thoracic Spine: A Case Report and Review of the Literature

Context: Osseous epithelioid hemangioma is uncommon, and reports of epithelioid hemangiomas of the spine are especially rare.

Study Design: Case report.

Findings: A 43-year-old male was referred to our department with progressive gait disturbance. CT scans showed a lucent mass in the vertebral body at the T3 level. MRI of the thoracic spine showed a strongly enhanced mass compressing the spinal cord. The patient underwent laminectomy from T2 to T4, debulking of the tumor, and posterior fusion from T1 to T5. After the operation, the patient's neurological status improved significantly, and he was able walk without assistance. Histological examination determined that the tumor was an epithelioid hemangioma. The patient was treated with 40 Gy radiation for local control of the tumor. The patient could walk without difficulty 12 months after the surgery.

Conclusion: This is a rare example of an epithelioid hemangioma that developed in the thoracic spine and compressed the spinal cord, and was treated successfully.     

Round Cell Sarcoma with EWSR1-PATZ1 Fusion in the Face of a Five-Year-Old Boy: Report of a Case with Unusual Histologic Features

Round cell sarcomas with EWSR1-PATZ1 fusion are rare polyphenotypic sarcomas that typically show both neural and myogenic differentiation on immunohistochemistry. The histology features lobular admixture of cellular fascicles of relatively monotonous spindle cells and small blue round cells separated by fibrotic stroma. The clinical behavior of EWSR1-PATZ1 sarcoma is uncertain currently with mixed outcomes reported even in cases with metastases. We herein report an additional case of EWSR1-PATZ1 fusion-related round cell sarcoma in the face of a 5-year-old boy with unusual histologic features of pale zones, rosette/gland-like structures and expression of epithelial markers. Fluorescent in-situ hybridization study (FISH) using EWSR1 breakapart probes was negative and molecular study with RNA sequencing was required to confirm the diagnosis. These findings highlight the diagnostic challenge and potential pitfall of FISH study in EWSR1-PATZ1 sarcoma. Further studies are required to increase the understanding of their behavior, morphologic spectrum and molecular features that will help devise new treatment strategies to these rare tumours.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12105-021-01285-w.     

Angiomyolipoma of the Nasal Cavity: A Distinct Entity. A Case Report and Literature Review

Angiomyolipoma (AML) is a mesenchymal tumor commonly found in the kidneys. Extra-renal AML is uncommon and especially rare in the nasal cavity. To our knowledge, fewer than five cases of nasal AML are reported in the United States. We present a case of nasal AML in a 65-year-old man with a history of rhinosinusitis and obstruction of the left nasal cavity. The lesion comprised of admixed spindled smooth muscle cells, mature adipose tissue, and thick-walled blood vessels. Smooth muscle differentiation of the spindled cells was confirmed by expression of smooth muscle actin. Surprisingly, melanocytic markers, such as HMB45, were negative. The histologic features and immunoprofile suggest that nasal AML is pathologically distinct from neoplastic AMLs/perivascular epithelioid cell tumors (PEComas) that typically occur in the kidney. We propose that nasal AML is a hamartomatous lesion rather than neoplastic. Although AML is rare in the nasal cavity, it should be considered in the differential diagnosis of clinically benign nasal masses.     

Primary Paraganglioma of the Parathyroid: A Case Report and Clinicopathologic Review

Paragangliomas are relatively uncommon neoplasms that arise in adrenal and extra-adrenal paraganglia of the autonomic nervous system. Parasympathetic paraganglioma develop predominantly in the head and neck. It is exceedingly uncommon to develop a primary intraparathyroid paraganglioma. There is only a single case report in the English literature. The information from the single previous case report (Medline 1960–2009) was combined with this case report. Our patient was a 69 year old woman who presented with a thyroid gland mass, with extension into the substernal space. The patient had a history of renal cell carcinoma removed 18 months before. At surgery, a thyroid lobectomy and a parathyroidectomy were performed. The parathyroid tissue showed a very well defined zellballen arrangement of paraganglion cells within the parenchyma of the parathyroid gland. The cells had ample basophilic, granular cytoplasm. The nuclei were generally round to oval with ‘salt-and-pepper’ nuclear chromatin distribution. There was a richly vascularized stroma. Mitotic figures, necrosis, invasive growth, and profound nuclear pleomorphism were absent. The neoplastic cells were strongly and diffusely immunoreactive with chromogranin, synaptophysin, CD56, and focally with cyclin-D1. The paraganglioma showed a delicate S-100 protein positive supporting sustentacular framework. Keratin, CD10, PTH, calcitonin and RCC markers were negative. The patient showed no stigmata of Multiple Endocrine Neoplasia (MEN) and has no paraganglioma in any other anatomic site. She is alive without any additional findings 12 months after surgery. Isolated paraganglioma within the parathyroid is rare, and should be separated from parathyroid adenoma, hyperplasia or metastatic disease to assure appropriate management.     

Hybrid Central Odontogenic Fibroma with Giant Cell Granuloma-like Component: Case Report and Review of Literature

Central odontogenic fibroma (COF) is a rare benign ectomesenchymal tumor of the jaws. Only 12 cases of COF with giant cell granuloma (GCG)-like lesion have been reported in the English literature. Here, we present a new case of COF epithelium rich type with a GCG-like component. Radiographically, this lesion presented as a well defined unilocular radiolucency in the body of the mandible. Histologically, the lesion showed a unique confluence of odontogenic epithelial rests with multinucleated giant cells (MNGCs) in a highly cellular fibrous connective tissue stroma, with osteoid and cementoid deposits. A distinct area showed the typical histological picture of each component separately. Immunohistochemical staining with pancytokeratin (CK) highlighted the odontogenic epithelial component merging with the GCG component throughout most of the lesion. The significance of GCG-like areas within COF is the reported increased risk of recurrence following curettage, possibly necessitating more aggressive therapy.     

Solitary Langerhans Histiocytosis of the Thyroid Gland: A Case Report and Literature Review

Langerhans cell histiocytosis (LCH) is a rare disease of antigen presenting cells, with an incidence rate of 4.0–5.4 per 1 million individuals. The most common endocrinological manifestation of classical LCH is associated with the posterior pituitary, presenting as Diabetes Insipidus. However, LCH can affect multiple organs and classification is based on the body system involvement. The disease is confirmed by electron microscopy or immunohistochemical reactivity of histiocytes to CD1a and/or S100. LCH rarely involves the thyroid gland, and management of such disease is controversial. Current literature documents 65 English language reported cases of LCH involving the thyroid gland. We present an unusual case of LCH of the thyroid gland, with variable diagnoses on fine needle aspiration (FNA) cytology, and literature review of all English reported cases.     

Intrasalivary Thymic Carcinoma: A Case Report and Literature Review

Ectopic thymic carcinomas are rarely diagnosed in the thyroid gland, let alone in extrathyroid tissues. In the currently available literature, only five cases of extrathyroidal malignancies with thymic differentiation have been reported as arising in the major salivary glands. A 69-year-old female presented with a slow-growing palpable mass in the left parotid gland. Fine needle aspiration biopsy suggested metastatic cancer, whereas core needle biopsy revealed high-grade squamous cell carcinoma. The patient underwent left radical parotidectomy with selective ipsilateral lymph node dissection and subsequent radiation therapy. The surgical specimen was taken for histopathological examination. Microscopically, the tumor resembled thymic carcinoma. It was composed of large nests of squamoid cells with smooth contours, focally with a syncytial growth pattern, and accompanied by abundant lymphocytes with reactive lymphoid follicles. This appearance resembled a micronodular thymic carcinoma with lymphoid hyperplasia. Moreover, the tumor displayed expression of squamous markers (p40 and p63) and markers of thymic carcinoma (CD5 and CD117). Therefore, the final diagnosis of intrasalivary thymic carcinoma was rendered. The molecular analysis including next-generation sequencing demonstrated no variants of the strong, potential, or unknown clinical significance. The patient remains disease-free at 1-year follow-up. In the current case, we comprehensively present a clinical, microscopic, molecular, and radiological picture of CD5-positive squamous cell carcinoma of the parotid. We postulate that similar cases should be designated as intrasalivary thymic carcinoma analogically to similar thyroid tumors. Our case and the limited literature data indicate they should be distinguished from conventional squamous cell carcinoma of major salivary glands due to their rather favorable prognosis.     

Merkel Cell Carcinoma of the Foot: Case Report and Review of the Literature

Merkel cell carcinomas are rare neuroendocrine tumors of the skin with highly malignant behavior. These tumors arise from Merkel cells, which are slowly adapting type 1 mechanoreceptors that occur in hair follicles and the basal layer of the epidermis. Merkel cell carcinomas are typically located in the dermis and frequently extend into the subcutaneous fat and lymphatics. This case report describes the magnetic resonance imaging and pathologic findings, surgical treatment, and postoperative results with review of the literature for Merkel cell carcinoma involving the foot of a 72-year-old man with a 2-month history of a painless, enlarging mass that interfered with his ability to wear a shoe.     

Primary Laryngeal Clear Cell Chondrosarcoma: Report of a Case and Literature Review

We present an example of clear cell chondrosarcoma (CCC) arising in the thyroid cartilage, and review the literature regarding this neoplasm in the larynx. This patient has no evidence of disease 3 years post hemilaryngectomy. Although rare and morphologically distinct, CCC follows the same benign clinical course as conventional laryngeal chondrosarcoma.     

颅内髓外造血组织瘤样增生1例及文献复习

目的探讨髓外造血组织瘤样增生的临床表现、诊断及治疗方法. 方法髓外造血组织瘤样增生1例,经开颅手术切除,经病理学检查得以确诊. 结果随访至今近一年,患者未见复发,生活如常.结论颅内髓外造血组织瘤样增生是一种罕见的特殊的良性增生性病变,CT、MRI等可定位诊断,其定性诊断依赖于病理学检查.治疗学上除按原发病治疗外,大的颅内病变需手术切除.     

Small Cell Carcinoma of the Bladder: A Case Report and Review of the Literature

Primary pure small cell neuroendocrine carcinoma of the bladder is a rare condition. It is an aggressive tumor with an average five-year survival rate of less than 10% as cited by multiple case reports. We report a 48 year-old male patient with primary small cell neuroendocrine carcinoma of the bladder who was treated with TUR-T, adjuvant carboplatin-based chemotherapy and radiotherapy. The patient is free of disease at the end of 30 months with a normally functioning bladder.     

肝脏血管周上皮样细胞瘤误诊1例报告并文献复习

目的介绍1例肝脏血管周上皮样细胞瘤(perivascular epithelioid cell tumor,PEComa)的诊疗过程,并进行文献复习。方法患者男性,26岁,因反复右上腹部疼痛不适3月,发现肝占位2周入院,术前诊断肝脏占位性病变,怀疑肝癌。遂于全麻下行腹腔镜下右肝后叶切除术,术后病理诊断为PEComa。结果患者痊愈出院,术后随访1月余无明显不适,无复发。结论PEComa是起源于间叶组织的一种少见肿瘤,由特征性的血管周上皮样细胞(perivascular epithelioid cell,PEC)组成。肝脏PEComa罕见,仅有文献个案报道。肝PEComa缺乏特征性的临床特点和体征,CT及MRI表现不典型,临床较易误诊为原发性肝细胞癌(hepatocellular carcinoma,HCC)。积极手术治疗,能取得较好疗效。