Dedifferentiated Liposarcoma With a Paraganglioma-like Histologic Pattern: A Case Report and Review of the Literature

2004 ◽  
Vol 128 (7) ◽  
pp. 788-791
Author(s):  
Dating Liu ◽  
Guillermo Quinonez ◽  
Steven Latosinsky

Abstract A 53-year-old man presented with a 4-month history of increasing abdominal discomfort and distension. A large retroperitoneal mass was found on imaging. Image-guided needle core biopsy demonstrated a poorly differentiated malignant neoplasm. A 30 × 32 × 33-cm soft tissue mass was removed. Microscopically, the tumor consisted of predominantly epithelioid malignant cells arranged in a paraganglioma-like growth pattern. Immunohistochemically, these cells were strongly positive for neuron-specific enolase. Stains for synaptophysin and chromogranin, however, were negative. There was no ultrastructural evidence of neuroendocrine differentiation. Adjacent sarcomatous areas were composed of spindled cells arranged in storiform and fibrosarcoma-like growth patterns. A small area of well-differentiated liposarcoma was identified, and a diagnosis of dedifferentiated liposarcoma was established. To the best of our knowledge, this represents the first reported case of dedifferentiated liposarcoma with a paraganglioma-like histologic pattern. A brief review focusing on the morphologic variations of dedifferentiated liposarcoma is also presented.

2020 ◽  
Vol 14 (1) ◽  
pp. 186-196
Author(s):  
Ahmed Ahmed ◽  
Umair M. Nasir ◽  
Paul Delle Donna ◽  
Vanessa Swantic ◽  
Shahida Ahmed ◽  
...  

Lung cancer is a common malignancy which is frequently found to metastasize to distant sites including bone, liver, and adrenal glands. There are rare reports of metastases to the gastrointestinal (GI) tract, with the duodenum being the most uncommon. We present a rare case of a poorly differentiated lung carcinoma metastasizing to the duodenum. This case enhances the medical literature as it provides additional distinct features to the clinical and histological presentation of metastatic lung carcinoma to the GI tract. A 61-year-old male with a history of poorly differentiated lung carcinoma presented with worsening dizziness, fatigue, and early satiety. He had extensive workup done in the past for hemoptysis including a computerized tomography scan of the chest which showed a new lobulated, apical lesion and hilar lymphadenopathy. He ultimately had a transthoracic fine-needle aspiration (FNA) of the mass and was later diagnosed with poorly differentiated lung carcinoma. On examination, the patient was noted to be pale, tachycardic, and hypotensive. The patient was noted to have an acute drop in his hemoglobin requiring fluid resuscitation, multiple blood transfusions, and evaluation with an esophagogastroduodenoscopy. He was found to have an oozing ulcer in the third portion of the duodenum whose biopsies showed poorly differentiated carcinoma with areas of neuroendocrine differentiation, similar to his lung biopsy results, which was consistent with metastatic lung carcinoma.


2020 ◽  
Vol 153 (6) ◽  
pp. 811-820 ◽  
Author(s):  
Kelsey E McHugh ◽  
Sanjay Mukhopadhyay ◽  
Erika E Doxtader ◽  
Christopher Lanigan ◽  
Daniela S Allende

Abstract Objectives INSM1 has been described as a sensitive and specific neuroendocrine marker. This study aims to compare INSM1 with traditional neuroendocrine markers in gastrointestinal neuroendocrine neoplasms. Methods Retrospective review (2008-2018) was used to retrieve paraffin-embedded tissue from 110 gastrointestinal neuroendocrine neoplasms and controls that was subsequently stained with INSM1, synaptophysin, chromogranin, CD56, and Ki-67. Results INSM1 was positive in 16 of 17 (94.1%) gastric, 17 of 18 (94.4%) pancreatic, 13 of 18 (72.2%) small bowel, 17 of 21 (81.0%) colonic, and 26 of 36 (72.2%) appendiceal tumors. INSM1 was positive in 58 of 70 (82.9%) well-differentiated neuroendocrine tumors, 17 of 20 (85.0%) poorly differentiated neuroendocrine carcinomas, 8 of 11 (72.7%) low-grade goblet cell adenocarcinomas (grade 1), and 6 of 9 (66.7%) high-grade goblet cell adenocarcinomas (grade 2/3). INSM1 sensitivity for neuroendocrine neoplasms (80.9%) was less than that of synaptophysin (99.1%), chromogranin (88%), and CD56 (95.3%); specificity was higher (95.7% vs 86.0%, 87.3%, and 86.0%, respectively). Conclusions INSM1 is a useful marker of neuroendocrine differentiation in gastrointestinal neuroendocrine and mixed neuroendocrine neoplasms. Compared with traditional neuroendocrine markers, INSM1 is less sensitive but more specific.


2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Eric Johannesen ◽  
Van Nguyen

Neuroendocrine tumors, distinguished from adenocarcinomas by their neuroendocrine differentiation, are the most common pediatric epithelial malignancy that most often occurs in the appendix. In 2010, the WHO classified neuroendocrine neoplasms into three grades based on morphology, mitotic count, and Ki67 proliferation index. A 15-year-old male with a history of anemia and failure to thrive was diagnosed with a well-differentiated neuroendocrine tumor in the jejunum that invaded into the subserosal soft tissue and metastasized to four lymph nodes. Pediatric neuroendocrine tumors frequently arise within hereditary tumor syndromes with pancreatic neuroendocrine tumors being the most common. Several studies also indicate an elevated risk of small intestinal neuroendocrine tumors in which children born to a parent with a history of neuroendocrine tumors in the small intestine have a significant increased risk of developing one.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Takao Natsuyama ◽  
Yozo Mitsui ◽  
Masato Uetani ◽  
Shigeyuki Ohta ◽  
Masafumi Inoue ◽  
...  

We report an extremely rare case of an alpha-fetoprotein- (AFP-) producing female primary urethral adenocarcinoma with neuroendocrine differentiation (NED). The patient was a 65-year-old woman with a 2-year history of urinary frequency and voiding difficulty. Enhanced computed tomography showed an approximately 3.0×5.0-cm mass around the proximal urethra and bladder neck. Of examined tumor markers, serum AFP was elevated (48.3 ng/mL), while others including carcinoembryonic antigen were within a normal range. Transurethral resection of the tumor led to a diagnosis of carcinosarcoma of the urethra, with a radical cystourethrectomy and ileal conduit formation subsequently performed. The pathological assessment was poorly differentiated adenocarcinoma in the urethra. Immunostaining showed tumor cells strongly positive for AFP. In addition, some cancer cells were positive for CD56, chromogranin A, and synaptophysin, indicating focal NED. The tumor was finally diagnosed as an AFP-producing urethral adenocarcinoma with NED. Serum AFP was immediately normalized after surgery and no sign of tumor recurrence has been noted 2 years postoperatively.


2000 ◽  
Vol 124 (4) ◽  
pp. 570-576
Author(s):  
Terence N. Moyana ◽  
Jim Xiang ◽  
A. Senthilselvan ◽  
Andrew Kulaga

Abstract Context.—The advent of panneuroendocrine markers has helped to better depict the heterogeneity of gastrointestinal carcinoids. Consequently, it has been proposed that these tumors constitute a histologic spectrum that includes well-, moderately, and poorly differentiated carcinoids. However, the reproducibility of this grading system and its prognostic importance have sometimes been called into question. Objective.—To investigate the potential utility of cell proliferation and oncoprotein markers in augmenting the histologic classification. Design and Setting.—Retrospective study; tertiary care teaching hospital. Methods.—Fifty-eight patients with 41 well-differentiated, 12 moderately differentiated and 5 poorly differentiated carcinoids from various topographic sites of the gastrointestinal tract were selected and immunostained for panneuroendocrine markers, MIB-1, p53, and bcl-2. Main Outcome Measures.—Degree of association between histologic grading, MIB-1, p53, and bcl-2 immunoreactivity and carcinoid metastatic behavior. Results.—The group comprised 30 males and 28 females whose mean age was 52.7 years (range, 14–81). Mean follow-up time was 85.8 months. All 58 patients tested positive for chromogranin A and/or synaptophysin. The group was divided into nonmetastatic (42/58, or 72.4%) and metastatic (16/58, or 27.6%) cases. Histologic grading tended to be associated with metastatic spread, but this occurrence of metastases did not attain statistical significance (P = .08). Positivity for MIB-1 (P = .004) and p53 (P = .04) was significantly associated with metastatic behavior, whereas bcl-2 was not (P = 0.63). Conclusions.—Although an organoid pattern and neuroendocrine immunophenotype help to define the spectrum of gastrotestinal carcinoids, this study suggests that the histologic grading of these tumors has some limitations with respect to predicting metastatic behavior. However, MIB-1 and p53 can compliment histologic grading as prognostic indicators in this regard while bcl-2 appears to be less useful.


2008 ◽  
Vol 45 (6) ◽  
pp. 905-909 ◽  
Author(s):  
A. R. Tucker ◽  
J. R. Smith

An 11-year-old cat with a retained testis was presented with a chronic history of dysuria and bladder atony. Medical therapy failed to alleviate the clinical signs. Contrast radiography demonstrated a diffusely narrowed urethra. During a celiotomy and prepubic urethrostomy, a retained testis, stenosed urethra, and irregularly enlarged prostate were observed. Histopathologic diagnosis was retained testis with a well-differentiated interstitial cell tumor, a poorly differentiated interstitial cell tumor, and marked squamous metaplasia of the prostatic epithelium with suppurative prostatitis. Neoplastic interstitial cells were immunoreactive for Melan A, consistent with reports of Melan A expression in steroid hormone-producing tissue. This is the first report of prostatic squamous metaplasia associated with testicular neoplasia in a felid.


2021 ◽  
Vol 59 (244) ◽  
pp. 1320-1322
Author(s):  
Sulochana Neupane ◽  
Sanam Dhakal ◽  
Shripad Walawalakar ◽  
Surya Bahadur Parajuli ◽  
Sulav Sapkota

Primary neuroendocrine carcinomas of the breast are rare of all breast carcinomas. They may be welldifferentiated, poorly differentiated, or invasive breast cancers with neuroendocrine differentiation. They are staged and treated similarly to conventional breast cancer. Herein, we report a case of invasive ductal carcinoma with neuroendocrine differentiation of the breast in a 73 years female with a history of breast lump initially in the lower inner quadrant of left breast and a month later, similar lump at the same site in right breast. Patient underwent Modified Radical Mastectomy bilaterally followed by adjuvant chemotherapy based on Carboplatin and Etoposide regimen.


2021 ◽  
Vol 10 (01) ◽  
pp. e42-e44
Author(s):  
Rajika Jindani ◽  
Michael A. Lopez ◽  
Tatiana P. Miquel ◽  
Erik Sylvin

Abstract Background Pulmonary epithelial–myoepithelial carcinoma (P-EMC) is an extremely rare, well-differentiated, and malignant neoplasm originating from submucosal bronchial glands in the lung. EMCs arise mainly in the salivary glands. Case Description This case represents an asymptomatic 78-year-old male with a remote 75-pack-year history of smoking who presents with a solitary endobronchial lesion, which is suggestive of a primary lung EMC, detected on annual screening chest computed tomography (CT) scan. Conclusion A recent review of literature reveals less than 50 documented cases of the pulmonary subtype of this tumor worldwide. We are reporting a unique case of robot-assisted pulmonary lobectomy for a P-EMC.


2020 ◽  
Vol 6 (2) ◽  
pp. 205511692097124
Author(s):  
Slavomίra Néčová ◽  
Susan North ◽  
Stephen Cahalan ◽  
Smita Das

Case summary A 15-year-old neutered male domestic shorthair cat presented with a 4-week history of dysphonia and reluctance to chew hard food. Oral examination revealed a mass lesion on the caudal soft palate. Biopsy was performed and histopathology with immunohistochemistry was most consistent with histiocytic sarcoma. CT of the head identified a discrete, left-sided, soft tissue mass lesion cranial to the tonsil with bilaterally symmetrical regional lymph nodes. CT of the thorax was unremarkable. Surgical removal achieved cytoreduction but not complete removal. Adjuvant chemotherapy with lomustine 10 mg (30 mg/m2) was initiated. The patient developed suspected pancreatitis 2–3 weeks postoperatively, so further chemotherapy treatment was discontinued, but supportive treatment with pain relief and appetite stimulants was provided. Three months postoperatively, the patient developed recurrent dysphonia, and oral examination revealed a suspicion of local recurrence. Confirmation of diagnosis (cytopathology or histopathology) was not obtained. Supportive treatment to maintain a good quality of life was continued and the patient was euthanased 6 months after diagnosis owing to progressive disease. Relevance and novel information Only a few reports have been published describing histiocytic diseases of cats. To our knowledge, this is the first report of a feline histiocytic sarcoma of the oral cavity. Therefore, histiocytic sarcoma should be considered as a differential diagnosis in feline patients with an oral mass, especially if histopathology suggests a pleomorphic and poorly differentiated sarcoma. Immunohistochemistry for the confirmation of cell line origin would be strongly recommended.


2019 ◽  
Vol 47 (10) ◽  
pp. 5312-5317
Author(s):  
Leyi Cai ◽  
Shuangxia Dong ◽  
Hua Chen

Tumours in the olecranon are rare. A 62-year-old man presented with a 3-week history of pain in his right elbow that was relieved using routine painkillers. Persistent pain resulted in a reduction of the range of motion in the elbow and pain in the extensor and flexor groups of muscles of the forearm, which resulted in the patient seeking medical assistance. Radiography showed a round, radiolucent focus in the depth of the distal ulna. Computed tomography imaging of the thorax demonstrated one solid soft tissue mass. Histological investigation showed the cancer was a poorly differentiated adenocarcinoma. The patient received local radiotherapy and systemic chemotherapy, but he died of his primary disease 3 months later.


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