A new form of alpha-chain disease with generalized lymph node involvement

An autopsy case of alpha-chain disease (ACD) clinically manifesting generalized lymph node swelling, slight splenomegaly and long-standing ichthyosiform skin eruptions, was reported. Autopsy revealed systemic superficial and profound lymph node swelling and slight splenomegaly, but little or no tumorous lesion in any part of the alimentary tract or pulmonary tissue. The histologic picture of the lymph nodes showed a diffuse monomorphic plasmocytic lymphoma, and there was tumor cell infiltration in the spleen and bone marrow. Immunohistochemistry demonstrated that the tumor cells contained IgA devoid of light chains, i.e. ACD protein. Immunoelectron microscopy revealed that this abnormal immunoglobulin was localized in the rough endoplasmic reticulum and perinuclear space. Persistent chronic inflammation with infiltration mainly of helper-inducer T cells were found in the skin and dermatopathic lymphadenopathy was confirmed in the lymph node biopsies. From these peculiar clinicopathological features, this case is considered to be a previously unknown form of ACD.     

Intraparenchymal pulmonary lipoma: pathologic-radiologic correlation of a rare presentation of a common neoplasm

We report a rare case of pulmonary intraparenchymal lipoma. Lipomas are benign adipocytic tumors, which are ubiquitous in distribution, particularly in the subcutis and soft tissue. Visceral lipomas, in particular, pulmonary lipomas, are rarely reported. Even rarer are intraparenchymal lipomas, such as this case, of which less than 10 have been reported in the medical literature. The radiologic (computed tomographic scan) findings of pulmonary lipoma may be somewhat difficult to evaluate. In this case, on initial review, the computed tomographic findings were not diagnostic, but retrospective analysis revealed attenuation values suggestive of an adipocytic lesion. A high index of suspicion and careful attention to attenuation values are therefore required for radiologic diagnosis. Excision is necessary for histologic confirmation, which is generally relatively straight forward, although admixture with fibrous tissue and some cytologic atypia may pose diagnostic challenges.     

Undifferentiated carcinoma with osteoclast-like giant cells: A pathologic-radiologic correlation of a rare histologic subtype of pancreatic ductal adenocarcinoma

Undifferentiated carcinoma with osteoclast-like giant cells (UC-OGC) is an exceedingly rare subtype of pancreatic ductal adenocarcinoma. Histologically, UC-OGC is characterized by three cell types namely, a neoplastic mononuclear cell component, non-neoplastic osteoclast-like giant cells, and a non-neoplastic mononuclear histiocytic component. The behavior of this tumor is unpredictable; but many patients survive many years after diagnosis. UC-OGC may have a better prognosis compared to conventional pancreatic adenocarcinoma due to its slower local spread, less aggressive nature, better response to surgical resection and/or chemotherapy, and fewer metastases. Due to likely differences in prognosis and significant impact on patient management, it is important to distinguish this subtype from other types of pancreatic adenocarcinoma. We report a case of a small (<1 cm) undifferentiated carcinoma with osteoclast-like giant cells of the posterior pancreatic body discovered incidentally on magnetic resonance image (MRI) scan of a middle-aged man. The radiologic and pathologic findings are presented along with a discussion of the differential diagnosis of this exceedingly rare entity.     

Inflammatory myofibroblastic tumor of the pancreas with regional lymph node involvement

Inflammatory myofibroblastic tumors (IMT) can be found in virtually any location of the human body. Histologically a mesenchymal aspect predominates and makes these mostly benign tumors apt to be erroneously diagnosed as a soft tissue sarcoma. Cases showing infiltrative growth and local recurrence further complicate the assessment. Localization of an IMT in the pancreas is extremely rare. Clinical investigations regularly lead to the putative diagnosis of a malignant tumor and only subsequent histological examination can establish the correct tumor classification. We present the case of a 62-year-old woman with IMT of the pancreas. Evidence of lymph node involvement has not yet been reported in this setting.     

Nivolumab induced pulmonary sarcoid-like granulomas with a concurrent pleural schwannoma: A pathologic-radiologic correlation of a rare condition mimicking metastatic melanoma

Nivolumab is an anti-PD-1 antibody. The mechanism of action of nivolumab is inhibition of binding between PD-1 and PD-1 ligand. This causes activation of antigen-specific T cells that were previously unresponsive to cancer cells. This unique mechanism of action attributes the widespread use of nivolumab for the treatment of a variety of neoplastic conditions. On the other hand, this mode of action is associated with adverse effects as well.Schwannoma, also called neurilemmoma, is a benign peripheral nerve sheath tumor. Pleural schwannomas are very rare and very few cases have been reported in the medical English literature so far.Herein, we report a very rare case of concurrent presence of Nivolumab induced pulmonary sarcoid-like granulomas along with primary benign pleural schwannoma in a 49-year-old male. He was diagnosed with malignant melanoma of the right upper arm for which he underwent surgery and was receiving adjuvant chemotherapy. He developed pneumonitis during chemotherapy, and on imaging multiple reticular and nodular interstitial infiltrates were seen along with an incidental pleural mass with a high suspicion for metastasis. Wedge biopsy of the interstitial infiltrates was done and they were found to be pulmonary granulomas related to the nivolumab therapy he was receiving. The patient underwent excision of the pleural mass which showed histopathological and immunohistochemical features of schwannoma. The two conditions are unrelated and rarely encountered simultaneously. The radiologic and pathologic correlation along with differential diagnosis of these conditions are discussed.     

累及淋巴结的肾脏血管平滑肌脂肪瘤一例

患者男,33岁,乏力,低热1年,左侧腰部阵发性绞痛反复发作1个月于2002年4月5日入院,体检：无发热、发冷,夜间盗汗及尿频,尿急、尿痛、尿血等病史。B超,X线及CT检查均提示：左肾占位性病变,肾细胞癌可能性大。实验室检查无异常。专科检查：双肾区对称无隆起,左肾区叩击痛,左输尿管走行区轻度压痛,膀胱区隆起无压痛。临床诊断;左肾肿瘤。     

Angiomyolipoma of the kidney with lymph node involvement.

A case of renal angiomyolipoma with regional lymph node involvement is added to 20 cases previously reported. This represents the 11th case reported in a non-tuberous sclerosis patient. The electron microscopic features of the tumor are discussed and correlated with the light microscopic findings.     

A rare case of secretory breast carcinoma in a male adult with axillary lymph node metastasis

Secretory breast carcinoma is a rare tumor originally described in children but occurring equally in adult population, especially in women. This unusual subtype has a generally favorable prognosis, although several cases have been described in adults with increased aggressiveness and a risk of metastases even death. So far, merely ten cases of secretory breast carcinoma with metastatic axillary lymph node in male were reported. Here, we describe the eleventh case, a 24-years-old male who presented with a painless mass in the right breast was diagnosed to be “secretary breast carcinoma”, and subsequently underwent modified radical mastectomy and adjuvant chemotherapy.     

Angiomyolipoma with regional lymph node involvement and long-term follow-up study

The first long-term follow-up study of a patient who had renal angiomyolipoma with regional lymph node involvement is reported. The absence of recurrence after 15 years favors the view that regional lymph node involvement represents a multicentric hamartomatous change and is not a metastasis from a malignant renal tumor.     

Primary pulmonary glomus tumor with contiguous spread to a peribronchial lymph node

Glomus tumors are uncommon soft tissue tumors. Rare occurrences in visceral organs including the respiratory tract have been reported. The vast majority of these tumors are biologically benign. We report a case of primary pulmonary glomus tumor with atypical features characterized by mild nuclear atypia, local infiltration, and contiguous spread to a peribronchial lymph node. The current literature is reviewed.     

Hepatic angiosarcoma mimicking sinusoidal obstruction syndrome/venoocclusive disease: a pathologic-radiologic correlation

We present a case of a 63-year-old man with liver dysfunction and biopsy findings of venoocclusive disease (VOD) who, at autopsy, was discovered to have multifocal hepatic angiosarcoma. After double lung transplantation, he initially presented with signs of liver failure and portal hypertension resulting in recurrent high-volume ascites. Clinically, VOD was considered, and tacrolimus was discontinued, due to its known association with VOD. This, however, did not result in clinical improvement, and computed tomography eventually revealed the development of multiple low-attenuating hepatic lesions over the course of several months. Biopsies of the masses and background liver demonstrated changes most consistent with VOD, characterized by sinusoidal congestion affecting the centrilobular areas with associated hepatocyte atrophy and dropout. A reticulin stain highlighted deposition of reticulin fibers within the sinusoids and central veins. Scattered sinusoidal atypical cells were identified; however, a definitive diagnosis of malignancy was not possible. He eventually passed away because of complications of liver disease. At autopsy, there were multiple firm, red-brown masses identified throughout both hepatic lobes. Upon histologic review, the masses were shown to be angiosarcoma. Away from the tumor, the liver also demonstrated features of VOD. It is likely that the histologic appearance of VOD in the background liver probably represents secondary changes due to injury to the hepatic sinusoids by the primary malignancy. We conclude that it is necessary to consider the possibility of unsampled vascular malignancy when hepatic masses are identified on imaging and histology is consistent with VOD.     

Small bowel haemangioma with local lymph node involvement presenting as intussusception

Gastrointestinal haemangiomas make up 0.05% of all intestinal neoplasms. They are sometimes multiple and usually present with pain, bleeding, and obstruction. An associated haemangiomatous change in regional lymph nodes has not been reported previously. A woman of 21 years presented with abdominal pain and vomiting. Abdominal ultrasound and computed tomography scan showed a lower abdominal mass. Laparotomy revealed a small bowel tumour causing an intussusception together with enlarged mesenteric lymph nodes. Pathological examination revealed a small bowel haemangioma with mesenteric node involvement. The pathogenesis of haemangiomatous involvement of lymph nodes is discussed. Hamartomatous change is the likely cause in this patient.     

A very rare case of synchronous primary lung cancer lymph node metastasis

A 76 year old patient presented with two synchronous primary lung tumours. One was identified as an adenocarcinoma and the second as an atypical carcinoid tumour. When reviewing the lymph node slides it was seen that one lymph node contained metastatic deposits from both primary tumours. Synchronous lung tumours are fairly rare occurrences, but even rarer is the finding of synchronous metastases to the same lymph node. The atypical carcinoid deposit was very subtle in appearance and could easily have been overlooked. This case demonstrates a useful learning point to not miss these rare and subtle findings as the resulting tumour staging was affected and may have implications for further patient management.     

Specific lymph node involvement in scleromyxedema: a new diagnostic entity for hypermetabolic lymphadenopathy

Scleromyxedema is a generalized skin disease mostly associated with monoclonal gammopathy. In its chronic course, it can lead to systemic disorders related to mucin deposits in organs. We describe here specific lymph node involvement, hitherto not reported in scleromyxedema. A 68-year-old man with a 1-year history of micropapular eruption and skin sclerosis involving the neck, trunk, hands, and face was diagnosed with scleromyxedema associated with IgG kappa monoclonal gammopathy. Enlarged mediastinal lymph nodes found on thoracic X-ray and computed tomography scan were hypermetabolic on positron emission tomography. Lymph node biopsy showed partial nodal infiltration by numerous fibroblasts surrounded by mucin and collagen deposits, the same being observed on the skin biopsy. Lymph node and skin lesions both improved after intravenous immunoglobulin and corticosteroid treatment. Lymph node involvement in scleromyxedema should be considered in the etiological diagnosis of hypermetabolic, enlarged lymph nodes, especially if monoclonal gammopathy is associated.