Two cases of monoclonal nodular pulmonary amyloidosis and review of the literature

Nodular pulmonary amyloidosis (NPA) is an uncommon pathology of insoluble protein depositing in pulmonary parenchyma. This localized pulmonary form of amyloidosis is most often found to contain combinations of kappa and lambda immunoglobulin light chain and immunoglobulin heavy chain proteins with a polyclonal lymphoplasmacystic infiltrate. Herein we present two cases of NPA of the rarely reported monoclonal (light-chain restricted) form with review of the literature and discussion of the clinical, radiographic, and histologic features of NPA

Retroperitoneal leiomyomas: A clinicopathologic and immunohistochemical study of 56 cases with a comparison to retroperitoneal leiomyosarcomas

Most retroperitoneal smooth muscle tumors are believed to be malignant, and leiomyomas are considered very rare. This study was undertaken to determine the clinicopathologic features and long-term follow-up of 56 tumors diagnosed as retroperitoneal leiomyomas (LM) or smooth muscle tumors with an uncertain malignant potential (SMTUMP) in an effort to correlate their behavior and clinicopathologic features. These tumors were compared with a series of 11 cases of retroperitoneal leiomyosarcomas (excluding gastrointestinal stromal tumors). Histologic slides and immunohistochemistry for SMA, desmin, S-100 protein, HMB45, CD34, C-KIT, estrogen (ER) and progesterone (PR) receptor proteins, and MIB-1 were analyzed. All tumors diagnosed as LM and all but one SMTUMP were well-differentiated smooth muscle tumors that lacked atypia and coagulative necrosis. There was \u3c1 mitosis per 50 high power field (HPF) in 38 tumors; no tumor had \u3e3 mitoses/50 HPF. Most tumors had a striking resemblance to uterine smooth muscle tumors with common hyaline change and trabecular patterns. There were 51 females and 5 males ranging in age from 25 to 79 years (mean 45 years, median 43 years). These tumors were typically large, with a mean size of 16.2 cm and weight of 1600 g. Immunohistochemically, all 35 tumors studied were positive for α-SMA, 30 of 35 tumors were positive for desmin, and all were negative for CD117, S100 protein, and HMB45 and all but one for CD34. Steroid receptors were commonly present: ER in 20 of 29 cases and PR in 26 of 31 cases in the tumors of female patients. MIB-1 score was \u3c2% in all of 28 cases. Long-term follow-up (mean 140 months) did not reveal metastases, but two patients had local recurrence; however, neither patient with recurrence demonstrated disease progression in follow-up. By contrast, all 11 leiomyosarcomas had at least mild atypia, and all were ER and PR negative, All cases had MIB-1-positive nuclei, but only four had \u3e10% nuclei positive. Four patients died of disease, four were alive with recurrence, and three had no evidence of disease. A group of benign leiomyomas can be identified among retroperitoneal smooth muscle tumors. Most of these tumors resemble uterine leiomyomas by histology and positive hormone receptors, and they seem to have a good long-term prognosis with a small potential for local recurrence

Multiple bony amyloidomas as an initial presentation of myeloma

Amyloidosis complicating multiple myeloma is an uncommon but well-recognized phenomenon. Multiple bone amyloidomas are rare as the initial presenting feature of myeloma. Solitary bone amyloidomas share common features with those of patients who have solitary plasmacytomas and progression to disseminated myeloma is common. We report a case of an elderly man who presented with extensive amyloid deposition in multiple plasmacytoma sites as well as evidence of amyloid in a fat pad aspirate but with none of the usual organ damage associated with systemic amyloidosis. This presentation is similar to a subset of patients said to have macrofocal myeloma. These patients are typically aged \u3c 40 years, have no bone marrow involvement, and have a good prognosis. This report may represent the first description of macrofocal myeloma associated with amyloid deposition in an older individual

A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature

BACKGROUND. Pancreatic lymphangiomas are rare benign tumors, of which only a few cases have been reported in the literature. In this study, the authors present a series of primary pancreatic lymphangiomas. METHODS. Cases of nonepithelial pancreatic cystic tumors (lymphangiomas) diagnosed between 1966 and 1994 were retrieved from the Endocrine Pathology Registry of the Armed Forces Institute of Pathology. Histologic features (in 10 cases) as well as histochemical and immunohistochemical studies (in 6 cases) were reviewed. Long term patient follow-up data were obtained in 9 cases. RESULTS. The patients included 8 females and 2 males ages 2-61 years (mean age, 28.9 years) at initial presentation. The tumors were circumscribed and occurred predominantly (in 6 of 10 cases) in the tail of the pancreas. The multicystic, serous, or chylous fluid-filled cystic tumors ranged from 3 to 20 cm (average, 12.7 cm) in greatest dimension. Histologically, the tumors consisted of multilocular cystic spaces of various sizes, lined by endothelial cells. The stroma contained smooth muscle and mature lymphocytes. Immunohistochemistry determined the endothelial lining cells to be factor VIII-R antigen and CD31 positive (in all cases tested) but usually CD34 negative. All patients for whom follow-up data were obtained (n = 9) were alive without evidence of disease an average of 7.2 years after initial diagnosis. CONCLUSIONS. Pancreatic lymphangiomas occur predominantly in females within a wide age range. Multilocular, fluid-filled cysts, with endothelial immunoreactivity for factor VIII-R antigen and CD31, are characteristic of these tumors. Complete surgical excision of these benign tumors resulted in excellent long term prognoses for all patients studied

Pancreatic endocrine tumors: Radiologic-clinicopathologic correlation

Pancreatic endocrine tumors (PETs) are primarily well-differentiated tumors composed of cells that resemble normal islet cells but that arise from pancreatic ductal cells. They are classified as functioning or nonfunctioning according to their associated clinical symptoms; insulinoma, gastrinoma, and glucagonoma are the most common functioning PETs. They also are classified according to their biologic behavior, although all PETs have malignant potential. Most are sporadic, but some are associated with familial syndromes such as multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, and neurofibromatosis type 1. At imaging, PETs typically appear as well-defined hypervascular masses, a finding indicative of their rich capillary network. Cystic change, calcification, and necrosis are common in large tumors, which are associated with a poorer prognosis and a higher prevalence of local and vascular invasion and metastases than are smaller tumors. Even when metastases are present, many well-differentiated PETs have an indolent course. Poorly differentiated PETs are rare and have an infiltrative appearance; patients with such tumors have a poor prognosis. Knowledge of the characteristic clinical, pathologic, and radiologic features of PETs is important in the evaluation and management of patients with a suspected clinical syndrome or a pancreatic mass

Gastrointestinal glomus tumors: A clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases

Glomus tumors usually occur in the peripheral soft tissues, but similar tumors have also been reported in the stomach and occasionally in the intestines. However, the relationship of these tumors to peripheral glomus tumors and gastrointestinal stromal tumors has not been fully clarified because previous series of gastrointestinal glomus tumors predate availability of immunohistochemistry. This clinicopathologic study examined 32 gastrointestinal glomus tumors. All but one of the tumors were located in the stomach and the remaining tumor was from the cecum. The tumors occurred with a strong female predominance (23 females and 9 males) and a median age of 55 years (range 19-90 years). The gastric tumors typically presented with gastrointestinal bleeding or ulcer-like symptoms, and 14 tumors had mucosal ulceration. Five tumors were incidental findings. The tumor sizes varied from 1.1 to 7 cm (median 2 cm), and most were located in the antrum. Histologically, the tumors typically had a solid pattern of sharply demarcated, round glomus cells with prominent, mildly dilated pericytoma-like vessels. Vascular invasion and focal atypia were relatively common (seen in 11 and 13 cases, respectively), and low mitotic activity (1-4 per 50 high power fields), was seen in 10 cases. Immunohistochemically, all tumors were positive for α-smooth muscle actin and calponin, and nearly all had a net-like pericellular laminin and collagen type IV positivity. All tumors were negative for desmin and S-100 protein. Three tumors had focal synaptophysin positivity, but none was positive for chromogranin. All tumors lacked KIT expression and the GIST-specific mutations in the c-kit gene. Follow-up revealed one patient death of metastatic disease to liver at 50 months; this tumor had 1 mitosis per 50 high power fields, but had spindle cell foci, mild atypia, and vascular invasion. Thirteen patients were well and alive after long-term follow-up. Gastrointestinal glomus tumors occur almost exclusively in the stomach, and they have a good overall prognosis, but a small, unpredictable potential for malignant behavior exists. These tumors are phenotypically similar to peripheral glomus tumors and differ from epithelioid GISTs

Sarcoidosis Presenting as Dorsal Wrist Mass: A Case Report

Patients with sarcoidosis have an indolent course in which the disease is not detected unless seemingly benign symptoms appear. Such was the case in a 42-year-old man who was referred to the orthopedic service for evaluation of a slowly enlarging mass over the left wrist without prior history of trauma. In this article, we will review the symptoms and histopathology of sarcoidosis with a particular focus on orthopedic manifestations of the disease. We believe that clinicians should be aware of these associations so that patients can be diagnosed and treated accordingly

Nonepithelial neoplasms of the pancreas, part 2: Malignant tumors and tumors of uncertain malignant potential

© RSNA, 2018. Almost all neoplasms of the pancreas are derived from pancreatic epithelial components, including the most common pancreatic mass, primary pancreatic ductal adenocarcinoma (PDAC). Nonepithelial neoplasms comprise only 1%–2% of all pancreatic neoplasms. Although some may arise directly from intrapancreatic elements, many originate from mesenchymal, hematopoietic, or neural elements in the retroperitoneal peripancreatic space and grow into the pancreas. Once these tumors reach a certain size, it can be challenging to identify their origin. Because these manifest at imaging as intrapancreatic masses, awareness of the existence and characteristic features of these nonepithelial neoplasms is crucial for the practicing radiologist in differentiating these tumors from primary epithelial pancreatic tumors, an important distinction given the vastly different management and prognosis. In part 1 of this article, the authors reviewed benign nonepithelial neoplasms of the pancreas. This article focuses on malignant nonepithelial neoplasms and those of uncertain malignant potential that can be seen in the pancreas. The most common malignant or potentially malignant nonepithelial pancreatic tumors are of mesenchymal origin and include soft-tissue sarcomas, solitary fibrous tumor, and inflammatory myofibroblastic tumor. These tumors commonly manifest as large heterogeneous masses, often containing areas of necrosis and hemorrhage. The clinical features associated with these tumors and the imaging characteristics including enhancement patterns and the presence of fat or calcification help distinguish these tumors from PDAC. Hematopoietic tumors, including lymphoma and extramedullary plasmacytoma, can manifest as isolated pancreatic involvement or secondarily involve the pancreas as widespread disease. Hyperenhancing paragangliomas or hypervascular metastatic disease can mimic primary pancreatic neuroendocrine tumors or vascular anomalies