Objective: To investigate the clinical, pathological and immunophenotypic features, molecular biology and prognosis of fibrin-associated large B-cell lymphoma (LBCL-FA) in various sites. Methods: Six cases of LBCL-FA diagnosed from April 2016 to November 2021 at the Beijing Friendship Hospital, Capital Medical University, Beijing, China and the First Affiliated Hospital, Wenzhou Medical University, Wenzhou, China were collected. The cases were divided into atrial myxoma and cyst-related groups. Clinical characteristics, pathological morphology, immunophenotype, Epstein Barr virus infection status, B-cell gene rearrangement and fluorescence in situ hybridization of MYC, bcl-2, bcl-6 were summarized. Results: The patients' mean age was 60 years. All of them were male. Three cases occurred in atrial myxoma background, while the others were in cyst-related background, including adrenal gland, abdominal cavity and subdura. All cases showed tumor cells located in pink fibrin clot. However, three cyst-related cases showed the cyst wall with obviously fibrosis and inflammatory cells. All cases tested were non germinal center B cell origin, positive for PD-L1, EBER and EBNA2, and were negative for MYC, bcl-2 and bcl-6 rearrangements, except one case with MYC, bcl-2 and bcl-6 amplification. All of the 5 cases showed monoclonal rearrangement of the Ig gene using PCR based analysis. The patients had detailed follow-ups of 9-120 months, were treated surgically without radiotherapy or chemotherapy, and had long-term disease-free survivals. Conclusions: LBCL-FA is a group of rare diseases occurring in various sites, with predilection in the context of atrial myxoma and cyst-related lesions. Cyst-related lesions with obvious chronic inflammatory background show more scarcity of lymphoid cells and obvious degeneration, which are easy to be missed or misdiagnosed. LBCL-FA overall has a good prognosis with the potential for cure by surgery alone and postoperative chemotherapy may not be necessary.
Humans ; Male ; Middle Aged ; Atrial Fibrillation ; Epstein-Barr Virus Infections ; Fibrin/genetics* ; Herpesvirus 4, Human/genetics* ; In Situ Hybridization, Fluorescence ; Lymphoma, Large B-Cell, Diffuse/pathology* ; Myxoma ; Proto-Oncogene Proteins c-bcl-2/genetics* ; Proto-Oncogene Proteins c-bcl-6/genetics*

Adult ; Cerebral Infarction ; diagnosis ; diagnostic imaging ; etiology ; Heart Atria ; diagnostic imaging ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Myxoma ; complications ; diagnostic imaging

Aggressive angiomyxoma is a rare mesenchymal tumor. To discuss the clinicopathological characteristics, treatment and prognosis of aggressive angiomyxoma, four cases of aggressive angiomyxoma of soft tissue in abdominopelvic cavity were collected from January 2015 to August 2017 in Peking University International Hospital. The clinical data, imaging examination, histopathological features, immunophenotype, therapy and prognosis were analysed. The related literatures were reviewed. All of the patients were adult females, age range from 27 to 49 years and mean 33 years. The clinical complaint was abdominal distention with no definite predisposing factor, or occasional physical-exam finding with no obvious discomfort. Three cases were primary and one case was recurrent. Typical layered or swirled structural sign was presented by CT and MRI scanning of three cases. All tumors located in the pelvic cavity, and attached to the uterus, vagina, rectum, bladder or ureter. One case was involved in the abdominal cavity simultaneously,adhesive to the spine, inferior vena cava and spleen. The gross appearance of tumors was from 5 to 22 cm in maximum diameter. The sectioned surfaces were soft, solid, white or yellow-gray, focally accompanied by edema, mucoid degeneration or cystic change. Microscopic observation showed that tumor cells were short spindle shaped and little atypical, the stroma was loose like edematous mucus or collagen, and the vessels were rich in thin and thick-wall. Partially the vessel wall expressed hyaline degeneration. Also tumors might infiltrate surrounding tissue, such as fat or nerve. The immunohistochemistry results of all cases were estrogen receptor and progesterone receptor diffusely moderate positive, Desmin and smooth muscle actin mostly positive, whereas CD34 expressed only in vessel and S-100 protein, CD117 and Dog1 all negative. All the tumors were complete surgical excision. During follow-up, one case recurred the second time. Our conclusions are the diagnosis of aggressive angiomyxoma is based on pathological morphology supplemented by immunohistochemistry, and the tumor may relapse after surgical resection.
Adult ; Desmin/analysis* ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Myxoma/pathology* ; Neoplasm Recurrence, Local ; Receptors, Estrogen/analysis*

We report the case of a 69-year-old woman who was diagnosed with intracardiac schwannoma without symptoms. Preoperative echocardiography and cardiac magnetic resonance imaging showed a mass attached to the interatrial septum. The initial diagnosis was a myxoma or a bronchogenic cyst. The tumor was successfully excised under cardiopulmonary bypass. However, the pathology of the excised tumor was consistent with schwannoma. We suggest that cardiovascular surgeons consider schwannoma to be a possible differential diagnosis for a mass close to the interatrial septum.
Aged ; Bronchogenic Cyst ; Cardiopulmonary Bypass ; Diagnosis ; Diagnosis, Differential ; Echocardiography ; Female ; Heart Neoplasms ; Humans ; Magnetic Resonance Imaging ; Myxoma ; Neurilemmoma* ; Pathology

Ear Canal ; pathology ; Ear Neoplasms ; diagnosis ; Humans ; Myxoma ; diagnosis ; Neoplasm Recurrence, Local ; diagnosis

OBJECTIVETo explore the pathological features and prognosis of appendiceal mucinous neoplasms (AMN) based on WHO classification 2010.

METHODSSeventy consecutive cases of AMN were classified into 5 groups according to WHO classification of digestive system tumors in 2010 including mucinous adenomas/cystadenoma (MA), low grade appendiceal mucinous neoplasms (LAMN), low grade pseudomyxoma peritoneum originated from appendix (PMP-L), invasive mucinous adenocarcinomas (MAC) and high grade pseudomyxoma peritoneum originated from appendix (PMP-H). Clinicopathological features, classification, treatment and prognosis of AMN were investigated retrospectively.

RESULTSThere were 11 cases of MA with neoplastic epithelium and mucin being defined in lumen and mucosa but without invasive lesions, and no relapse or death was found. In 41 LAMN cases, mucin was found in submucosa, muscularis proparis, or serosa of appendix, no or only scant mucinous epithelium with low grade dysplasia presented in mucinous pools in most cases (39/41). Among 41 LAMN cases, 3 developed relapse or PMP-L, and no death was observed. In 7 PMP-L cases, low grade dysplastic mucinous epithelium in mucinous pools could be found easily in 3 cases and was very scanty in 4 cases, with 1 relapse and 1 death. Eleven invasive carcinomas were found, including 7 MAC cases and 4 PMP-H cases, with local high grade dysplastic epithelium at least. In these invasive lesions, 4 cases recurred and 3 case died (including 2 recurred cases above). MA and LAMN were both non-invasive neoplasms histologically, however, PMP-L, MAC and PMP-H were regarded as adenocarcinomas according to their biological behavior.

CONCLUSIONAMN displays a relatively homogeneous group of neoplasms that pursues a predictable clinical course based on their nature, so it is necessary to diagnose and administrative accurately with consistently standards for these neoplastic entities.

Adenocarcinoma, Mucinous ; pathology ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Appendiceal Neoplasms ; classification ; pathology ; Child ; Female ; Humans ; Male ; Middle Aged ; Myxoma ; pathology ; Prognosis ; Young Adult

Unusual presentation of localized gingival enlargement associated with a subjacent tumoural pathology is reported. The patient was a 55-year-old black male, whose chief complaint was a progressive gingival overgrowth for more than ten years, in the buccal area of the anterior left mandible. According to the clinical features and the radiological diagnosis of odontogenic keratocyst, a conservative surgery with enucleation and curettage was performed. Tissue submitted for histopathological analysis rendered the diagnosis of odontogenic myxoma. After 12-month of follow-up, no evidence of recurrence was found. Clinicians should be cautious when facing any gingival enlargement to avoid diagnostic pitfalls and to indicate the appropriate treatment.
Diagnosis, Differential ; Gingival Overgrowth ; etiology ; pathology ; Humans ; Male ; Mandibular Neoplasms ; complications ; pathology ; surgery ; Middle Aged ; Myxoma ; complications ; pathology ; surgery ; Odontogenic Tumors ; complications ; pathology ; surgery

We report the case in a 72-year-old man who presented with a right inguinal mass and with a one month history that was initially interpreted as an inguinal hernia. Ultrasonography (US) and computed tomography (CT) demonstrated a right inguinal mass, including myxoid and fat component, extending from the right spermatic cord to the right inguinal subcutaneous layer. Mass excision was performed, and the diagnosis turned out to be angiomyxolipoma. Angiomyxolipoma is a rare tumor and the preoperative diagnosis of this disease is very difficult. However, angiomyxolipoma of the spermatic cord should be considered in the differential diagnosis in patients with an irreducible inguinal mass. Imaging diagnosis, such as US and CT may help to make a preoperative diagnosis.
Aged ; Angiolipoma/pathology/*radiography/*ultrasonography ; Hernia, Inguinal/ultrasonography ; Humans ; Male ; Myxoma/pathology/*radiography/*ultrasonography ; Spermatic Cord/*pathology/radiography/ultrasonography ; *Tomography, X-Ray Computed ; *Ultrasonography, Doppler

Adult ; Female ; Humans ; Middle Aged ; Myxoma ; pathology ; surgery ; Vulvar Neoplasms ; pathology ; surgery

Aggressive angiomyxoma is an uncommon mesenchymal myxoid tumor that is characterized by slow growth and frequent local recurrence. It is currently regarded as a nonmetastasizing tumor. We describe a case of recurrent aggressive angiomyxoma with invasion into the veins including the inferior vena cava and the right atrium and with pulmonary metastases. Our case, together with those unusual cases documented in previous reports, may lead to a reappraisal of the nature of aggressive angiomyxoma.
Adult ; Biopsy ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Lung Neoplasms/*secondary/surgery ; Myxoma/*diagnosis/pathology/surgery ; Radiography, Interventional ; Tomography, X-Ray Computed ; Vascular Neoplasms/*diagnosis/pathology/surgery ; Vena Cava, Inferior/pathology/surgery