Multiple Nodular Masses Research Articles

Primary Uterine Alveolar Soft Part Sarcoma in a Postmenopausal Woman: Histopathologic and Immunohistochemical Characteristics of a Rare Case.

Primary uterine alveolar soft part sarcoma (ASPS) is a rare, indolent mesenchymal malignancy with less than 40 patients documented in the literature. We report an example of ASPS in a 61-year-old postmenopausal woman. Macroscopically, the uterus showed multiple nodular masses. Microscopic examination revealed tumor arranged in nests and alveolar pattern. The tumor cells were moderately to markedly pleomorphic, epithelioid to polygonal, with eccentrically placed nuclei, vesicular chromatin, prominent macro-nucleoli, and moderate to abundant eosinophilic cytoplasm. PAS-positive and diastase-resistant intracytoplasmic crystals were also seen in some tumor cells. On immunohistochemistry, the tumor cells showed diffuse positivity for vimentin and nuclear positivity for TFE3, a surrogate marker for ASPS. These were negative for SMA, desmin, CD10, h-caldesmon, cyclin D1, EMA, Melan A, and CD34. SMARCB1 expression was retained. Based on the histopathology and IHC, a final diagnosis of uterine ASPS was rendered. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare tumors. Knowledge of the characteristic histopathologic and immunohistochemical features can help accurately diagnose such rare sarcoma in an uncommon site with an unusual age.

P16.01.A THE CASE OF AGGRESSIVE DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR IN ADULT TREATED WITH CRANIOSPINAL IRRADIATION AND TEMOZOLOMIDE

Abstract BACKGROUND Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare tumor, which was introduced as a separate entity in the 2016 World Health Organization classification of central nervous system tumors. It is most common in pediatric age, but very rare in adults. Literature demonstrates limited reports regarding treatment strategy in adult. We present a case of young female affected by DLGNT with aggressive behavior. MATERIAL AND METHODS A 28-year-old woman presented with severe headache and vomiting. Brain MRI showed diffuse leptomeningeal enhancement with hydrocephalus. At first, she was under suspicion of tuberculosis meningitis with mild increased protein level in CSF study. Her symptom rapidly improved after ventriculoperitoneal shunt and she started anti-tuberculosis medication. Despite 8-month of medication, her neurological status gradually deteriorated with paraparesis and follow-up MR images showed progression of disease. She underwent open biopsy of superficial brain lesions. RESULTS Intraoperatively, multiple nodular masses were attached inner side of dura mater and diffuse adhesion on subarachnoid space of brain surface. Histological examination revealed small round cells with oligodendroglial-like aspects. Immunohistochemistry showed positivity for Olig2, synaptophysin and S100. Chromosome 1p deletion was confirmed by FISH. Next-generation sequencing profile disclosed mutation of H3K28M, FGFR1 and ATRX. Diagnosis was consistent with DLGNT. She received craniospinal irradiation of 36 Gy in 20 fractions, then started chemotherapy with temozolomide (TMZ) at a dose 200mg/m2/day for 5 days in 4 weeks. After 4 cycles of TMZ, MR images demonstrated stable disease. CONCLUSION DLGNT is a very rare tumor in adult. Diagnosis needed high level of suspicion and histological confirmation. We present a very rare adult DLGNT patient with aggressive clinical features who showed stable state treated with craniospinal irradiation and TMZ chemotherapy.

Visceral stromal sarcoma in a dog: Case report

Visceral stromal sarcoma is a tumor that may cause no symptoms and, when it does, symptoms are unspecific. The tumor is usually an incidental finding, as it is detected while treating unrelated conditions. In this study, we aimed to highlight the importance of complementary exams in diagnosing stromal sarcoma in dogs. A nine-year-old Lhasa Apso dog was admitted into a veterinary hospital with a distended abdomen and decreased defecation. Ultrasound and hematological exams were requested. The hematology test revealed thrombocytosis, neutrophilia, and monocytosis. The ultrasound exam showed the spleen with increased dimensions. Presence of a neoformation occupying the entire left and right mesogastric abdominal topography was observed, apparently originating in the splenic tail, with irregular and coarse shape, mixed echogenicity, vascularized in some portions, and with cavitary areas and irregular walls in others, with anechoic content and echogenic dots in suspension measuring across the vascularized and cavitary portions. Associated with the formation, the mesentery and peritoneum were visible with a slight increase in echogenicity and coarse echotexture. These findings suggested that initial treatment should be surgical, which confirmed that the tumor did not originate in the spleen but in the mesentery. The tumor was removed, and an end-to-end anastomosis was performed at the site the tumor was adhered to. The tumor was taken for a biopsy and multiple nodular mass fragments were found, together measuring 10.0 × 9.0 × 2.5 cm. The inner surface of fragments of the fragments was observed to be whitish-beige, with yellowish areas, smooth and irregular. Biopsy confirmed a diagnosis of visceral stromal sarcoma and the patient was referred to an oncologist. Surgery was the most appropriate treatment in this case due to the size of the tumor, which could compromise the function of other systems. The complementary exam was critical in determining the diagnosis and deciding on the best course of action. In veterinary clinics where the complementary exams are not available, this case could have gone unnoticed.

What is your diagnosis? Urine from a dog.

What is your diagnosis? Urine from a dog.

Eumycotic mycetoma in a young girl from Sokoto, Nigeria: A rare and unusual presentation

Introduction: A typical presentation of Mycetoma is not uncommon although clinical manifestations might be misleading leading to delay in diagnosis, treatment and consequently leading to poor prognosis. Mycetoma can have a fungal or bacterial etiology and manifestation is usually that of a disfiguring subcutaneous infection that can affect any part of the exposed body. We are reporting a case of Mycetoma in an eleven years old girl that occurred in parts of the lower abdomen, perineum and gluteal region that was initially thought to be a soft tissue sarcoma or disseminated tuberculosis. Case Presentation: An eleven years old girl presented to Usmanu Danfodiyo University Teaching Hospital, Sokoto with lower abdominal mass and multiple nodular masses with discharging sinuses on the upper part of the right thigh, perineum and gluteal region of six months duration. Swellings started as multiple small boils that subsequently started discharging from various points. Patient usually fetches firewood in the forest for her parents as her routine house chores and she remembered an incident where she had pricks from thorns in the bush around her lower thigh and perineum. On examination she was chronically ill looking, in painful distress, with bilateral inguinal lymphadenopathy. She had nodular lesions of varying sizes ranging from 1x1cm to 4x4cm, tender, involving the upper part of the thigh bilaterally, but more on the right, lower abdomen, labia and gluteal region. Some of the lesions had hyper-pigmented sinuses discharging mucopurulent fluid, with areas of soft tissue swelling around the lower abdomen and upper right thigh extending to the leg. Patient was observed to walk with a limp gait. Management and Outcome: An initial diagnosis of soft tissue infection to rule out soft tissue sarcoma and disseminated tuberculosis (abdomen and lymph nodes) and deep tissue mycosis was made. However, with further investigations and reviews by the medical microbiologist and anatomic pathologist, along with bacteriologic, and mycologic studies of swab samples and aspirate and tissue biopsy for Histology revealed an eumycotic mycetoma. She received Ketoconazole and Trimetoprim-sulphametoxazole. She responded significantly as lesions reduced in sizes, abdominal swelling and leg swelling reduced with closure of discharging sinuses. Patient could walk with some resolution of the limp. Repeat abdominal ultrasound scan showed resolution of initial findings. She spent four weeks in the hospital and was discharged. On subsequent follow-up; she was walking without any limp and lesions were healed with some scar and few areas left to dry up. Further follow-up visits after one month and three month showed progressive healing and complete resolution of lesion respectively. However. Patient was however lost to further follow-up which would have enabled monitoring as to any reoccurrence or not. Conclusion: We presented a case of a young girl with an abnormal presentation of eumycotic mycetoma. Patient achieved near cure on medications without the need of surgery due to an excellent multidisciplinary approach between pediatricians, clinicians, clinical microbiologists and anatomic pathologists. Keywords: Mycetoma, Eumycetoma, Paediatrics

Molecular Pathological Differences in Global Gene Expression between Two Sustained Proliferative Lesions, Nodular Regenerative Hepatocellular Hyperplasia and Hepatocellular Adenoma, in Mice.

Long-term exposure to piperonyl butoxide (PBO) induces multiple nodular masses along with hepatocellular tumors in the liver of mice. The histopathological features of the nodules led to our diagnosis of nodular regenerative hepatocellular hyperplasia (NRH). However, because of the lack of data on the biological characteristics of NRH, whether this lesion is truly nonneoplastic remains unknown. In this study, the molecular characteristics of NRH were compared with those of hepatocellular adenoma (HCA) by global gene expression analysis. Six-week-old male ICR mice were fed a diet containing 6,000 ppm PBO for 43 weeks to induce NRH and HCA development. Complementary DNA microarray analysis was performed using messenger RNA extracted from NRH and HCA frozen sections collected by laser microdissection. Hierarchical cluster analysis showed that all NRH samples clustered together but were separate from the HCA cluster. Pathway analysis revealed activation of the cell cycle and Delta-Notch signaling in both lesions, but the latter was more upregulated in HCA. Downregulation of cytochrome p450 enzymes was observed in NRH, but not in HCA. These results imply that NRH differs from HCA in terms of not only morphological but also molecular characteristics.

The CT and MR imaging characteristics of juvenile xanthogranulomatosis in children

Objective To analyze the CT and MR imaging characteristics of the subcutaneous and organ lesions of uvenile xanthogranuloma (JXG) in children. Methods The CT and MR images of the head, chest, abdomen and subcutaneous soft tissue of 11 patients (6 females and 5 males, aged 2 months-12 years old, median age 8 months old) with JXG from 2006 to 2018 were collected and retrospectively analyzed. Eight patients underwent CT scan and/or contrast-enhanced CT scan, and 7 patients underwent MRI and/or MRI and contrast-enhancement MRI. Results In 11 cases of JXG, 5 cases were classified as simple skin type, involving the skin and deep soft tissue in the right posterior neck, the right frontal and parietal parts of the head, the nose root, and the fat layer and the deep muscle in the extremities. The lesions were the subcutaneous soft tissue nodules or mass showing isodensity on CT or high signal on T2WI. And the other 6 cases were classified as systemic type whose lesions involved one or more than one part except the skin. In the 6 cases, 3 cases involved the central nervous system, including the pituitary gland, cerebral hemisphere, cerebellum, brain stem, basal ganglia, thalamus, optic chiasma and spinal cord. The lesions of the pituitary gland were manifested as pituitary enlargement, thickening of the pituitary stalk, and disappearance of high signal on T1WI in the neurohypophysis. The brain parenchyma, the spinal cord and the optic chiasma showed multiple flake or nodular masses, which were iso/low signal on T1WI, low/high signal on T2WI, diffusion restricted, and enhanced markedly. Two cases involved lung tissue, and their lesions showed diffuse or scattered multiple cloudy shadow, reticular opacity, strip and small nodule shadow on chest CT. There was pleural effusion in one case. Two cases involved the livers and 2 cases involved the kidneys, showing multiple nodules with low signal on T2WI and slight enhancement. One case involving the pancreas showed a low density in the head of the pancreas and slight enhancement on CT. One case involving the greater omentum, chest wall and peritoneum showed thickening of the corresponding tissues on CT. Conclusions Subcutaneous and systemic lesions of JXG show some characteristics on CT and MRI images. The main manifestations are single or multiple nodulars, which are diffusely distributed and vary in size in subcutaneous soft tissue and various organs. The combination of imaging appearances, clinical manifestations and biopsy has important clinical significance for the correct diagnosis and evaluation of JXG. Key words: Granuloma; Tomography, X-ray computed; Magnetic resonance imaging

Clinical characteristics and treatment strategies of prostate mucinous adenocarcinoma: the multi-center summary of 36 cases

Objective To review the clinical characteristics of prostate mucinous adenocarcinoma cases and update literatures , and recommend the corresponding clinical treatment strategy. Methods From October 2010 to March 2018, 36 cases of prostate mucinous adenocarcinoma were involved from 5 urinary centers in China, including 9 cases from Shanghai Changhai Hospital, 4 cases from Wuhan Tongji Hospital, 13 cases from Shanghai Renji Hospitals, 8 cases from the First Affiliated Hospital of Nanjing Medical University, and 2 cases from Sichuan West China Hospitals. The patients′ age were (66.8±7.2) years (53-83 years) and the median PSA was 22.89 ng / ml (2.67-1786 ng / ml). Prostate biopsy confirmed Gleason score 3+ 3 points in 6 cases, 3+ 4 points in 9 cases, 4+ 3 points in 5 cases, 8 points in 11 cases, and 9 to 10 points in 5 cases. According to D′Amico risk stratification, 2 patients were in the low-risk group, 9 in the intermediate-risk group, and 25 in the high-risk group. Eight cases underwent radical retroperitoneal prostatectomy, 13 cases underwent laparoscopic radical prostatectomy, and 12 cases underwent robotic laparoscopic radical prostatectomy. Twenty-three cases underwent pelvic lymphadenectomy, including 12 cases of bilateral obturator lymph node dissection, and 11 cases of bilateral obturator+ intraorbital+ para-vascular para-aortic lymphadenectomy. Results All 36 operations were completed successfully.Twenty-three cases underwent pelvic lymphadenectomy, including 12 of bilateral obturator lymph node dissection, and 11 of bilateral obturator, intraorbital, and para-aortic lymphadenectomy. Pathological examination showed 9 cases of prostate mucinous adenocarcinoma, 26 cases of mucinous adenocarcinoma with acinar adenocarcinoma, and 1 case of mucinous adenocarcinoma with neuroendocrine and immunohistochemical positive of MUC2(+ ). Among 33 cases undergoing radical surgery, the pathological stage of ≤T2b in 12 cases (36.3%), T2c in 7 cases (21.2%), T3a in 7 cases (21.2%), T3b in 6 cases (18.2%), and T4 in 1 case (3.0%). Four cases had positive pelvic lymph nodes and 9 cases had positive margin. The median follow-up period was 26 months (6-48 months). The biochemical recurrence occurred in 6 patients one year after surgery, including 3 cases in the intermediate-risk group and 3 cases in the high-risk group. Six cases with postoperative biochemical recurrence and 19 cases with PSA>0.2 ng/ml after radical or palliative resection underwent adjuvant androgen deprivation therapy(ADT), no postoperative adjuvant radiotherapy or chemotherapy was administered, and 4 cases progressed to castration-resistant prostate cancer. Four cases with CRPC were in the high-risk group and had underwent radical surgery, and the median period progressed to CRPC was 26 months(3-37months)with 2 cases of death. However, there was no significant difference in the rate of biochemical recurrence and the incidence of CRPC in the low-risk group, the intermediate-risk group and the high-risk group. In addition, 2 cases had metastases, with pelvic MRI presenting pelvic multiple nodular mass in one case which was consistent with recurrence and metastasis at the 5th month after radical surgery, and pathological examination presenting the mucinous adenocarcinoma being neurosecretory in another case and mestastasis being detected on glans at the 3rd months after radical surgery. The recovery rate of urinary continience at 6 and 12 months after radical surgery was 86.2%(31/36) and 89.7%(32/36) respectively. Conclusions Prostate mucinous adenocarcinoma is a variant of acinar adenocarcinoma. This study clarifies prostate mucinous adenocarcinoma of Chinese patients with high Gleason scores, advanced pathological stage, variant in prognosis, and prone to recurrence and metastasis. For treatment strategy, the low-risk and intermediate-risk mucinous adenocarcinoma is recommended undergoing radical surgery, and the prognosis maybe good. High-risk mucinous adenocarcinoma could treated with radical surgery or palliative surgery with adjuvant ADT, and most high-risk patients can benefite, with a small number of poor prognosis. Key words: Prostatic neoplasms; Mucinous adenocarcinoma; Biochemical recurrence; Castration-resistant

Disseminated Peritoneal Leiomyomatosis and Metastatic GIST: Differential Diagnosis Dilemma Regarding Multiple Nodular Serosal Lesions and Management

We aim to discuss cases with gastrointestinal stromal tumor and leiomatosis peritoneal dissemeniata LPD regarding their diagnosis and surgical management with pitfalls related. We also aim to focus on management of multiple nodular serosal lesions. Two cases are presented. Both were diagnosed as advanced ovarian cancer on CT and ultrasound imaging by multiple nodular peritoneal lesions, omental cake and adnexal masses. Tumor markers were normal in both cases. Both were scheduled for exploratory laparotomy. One patient diagnosed with neurofibromatosis type I. Midline laparotomy was done for one case revealing normal adnexa with multiple fibroid uterus, peritoneal solid masses with large mesenteric and omental masses related to the small intestine. Total hysterectomy with bilateral salpingo-oophorectomy, total omentectomy and resection anastomosis for the small intestine with removal of macroscopic lesions were done. Midline line laparotomy was done for the second case revealing free adnexa with subserous vascular fibroids and omental fibroid solid masses. Total hysterectomy with bilateral salpingo-oophorectomy and total omentectomy and removal of macroscopic peritoneal lesions seen were done. The second case was diagnosed as Disseminated Peritoneal Leiomyomatosis, and the other case was diagnosed with multiple uterine fibroid and metastatic GIST on histopathology. Disseminated Peritoneal Leiomyomatosis and metastatic GIST can be similar on imaging and operative findings. Also they are similar to other lesions especially peritoneal carcinomatosis as in advanced ovarian cancer. These pathologies should be kept on mind when encountering multiple solid nodular masses in the visceral and parietal wall peritoneum and the omentum on imaging and intra-operative especially with normal associated tumor markers of ovarian cancer as serum CA125. Surgical and postoperative management differ according to the pathology. These pathologies should be confirmed by histopathology and immunohistochemistry. Frozen section is highly needed in these cases to optimize management.

Metastatic T-cell lymphoma of unknown primary origin in a dog (Canis lupus familiaris)

Canine lymphoma is a hematopoietic neoplasm that can affect different tissues. Its classification is performed according to cellular morphology, clinical stage, immunophenotyping, and anatomical location. However, in some cases, an oncopathy might be in an advanced stage with a plain organic spread, making it impossible to determine the initially affected tissue, which is a rare situation in animals. Thus, a case of metastatic lymphoma, of unknown primary origin, was described in a four-year-old Rottweiler bitch with multiple nodular subcutaneous proliferations. Hematological, biochemical, thoracic radiography, and abdominal ultrasound tests were performed, as well as a cytopathological examination of subcutaneous lesions and bone marrow. Hematologic findings determined anemia, lymphopenia, and thrombocytopenia. The thoracic radiography revealed multiple nodular masses. Cytopathological examination revealed infiltration of malignant lymphocytes into the subcutaneous nodules and into the bone marrow. The dog was euthanized and submitted to necroscopic examination, in which multiple nodules were found in the subcutaneous region, lung, pericardium, gastrointestinal tract and in the skeletal musculature, in addition to lymphadenopathy. Fragments of the affected structures were collected for later histopathological and immunohistochemical evaluation. Histopathological examination revealed the presence of neoplastic cells compatible with diffuse lymphoma, which was positively immunolabelled for T-cell lymphoma. Based on clinical-pathological findings and other complementary tests, a diagnosis of diffuse T-cell lymphoma of unknown primary origin was made. In order to classify hematopoietic neoplasms, it is essential to perform different laboratory tests. However, determination of origin becomes difficult when such an oncological disease presents an advanced stage.

Primary hepatic neuroendocrine tumors: multi-modal imaging features with pathological correlations

BackgroundPrimary hepatic neuroendocrine carcinomas (PHNECs) are rare and asymptomatic, and are therefore difficult to distinguish radiologically from other liver carcinomas. In this study, we aimed to determine the computed tomography (CT), magnetic resonance imaging (MRI), and digital subtraction angiography (DSA) features of PHNECs.MethodsA retrospective analysis of 11 patients with pathologically proven PHNECs was performed from January 2009 to September 2014. The CT, MRI, and DSA image features were analysed.ResultsTen of the eleven patients exhibited two or more lesions, and one patient exhibited a single lesion. Abdominal CT of 8 cases revealed multiple round or oval-shaped masses with well-defined borders, which were heterogeneous and hypodense on precontrast CT images. Significant diffuse heterogeneous enhancement was observed during the arterial phase in 8 cases, and the enhancement was slightly higher than the attenuation of the surrounding normal liver parenchyma and indistinct edges of small lesions during the portal phase. Well circumscribed (11 cases), lobulated (5 cases) or multiple nodular masses (4 cases), nodule (1 case) and irregular masses (1 case) of high signal intensity were observed on T2WI and DWI of MR images. The masses were well circumscribed, heterogeneous, and hypointense on T1WI, with significant enhancement of the solid carcinoma portion in the early arterial phase and continued enhancement in the portal venous phase. Characteristic lobulated or multiple nodular masses were observed in MRI. DSA showed multiple hypervascular carcinoma-staining lesions with sharp edges in the arterial phase.ConclusionThe CT, MRI, and DSA images of PHNECs exhibit specific characteristic features. Appropriate combinations of the available imaging modalities could therefore optimize the evaluation of patients with PHNECs.

Lymphome du MALT pulmonaire et syndromes paranéoplasiques

Primary pulmonary lymphoma is a rare disease; diagnosis is often delayed because of atypical clinical presentation and slow progression. A 42-year-old woman consulted because of haemoptysis. Chest CT-scan showed multiple nodular calcified masses. A lung biopsy led to the diagnosis of pulmonary amyloidosis with pulmonary MALT lymphoma (mucosa-associated lymphoid tissue). The patient developed two paraneoplastic syndromes: a hypertrophic osteoarthropathy and mucinosis. Multiple nodular amyloidosis can be a mode of presentation for pulmonary lymphoma. Paraneoplastic syndromes must be systematically considered and can help in early diagnosis of the disease and its relapse.

Pyogenic Liver Abscesses: Incriminating Perforated Appendicitis

We report a case of a 26 year old male with no prior medical history who presented with nausea, vomiting, abdominal pain, diarrhea and fevers for 3 weeks. Diarrhea was watery, non-bloody, occurring 2-3 times daily, associated with intermittent bilious vomiting. The patient admitted to anorexia with 15 pound unintentional weight loss over this time period. Abdominal pain was sharp, right upper quadrant, non-radiating, and progressively worsening. He denied any recent travel or sick contacts. On exam, the patient was febrile and tachycardic, with right upper quadrant tenderness. There was no rebound or tenderness at McBurney's point, and psoas and obturator signs were negative. Laboratory investigation revealed a WBC 16,500cells/mcL, AST 111U/L, ALT 74U/L, ALP 146U/L, total bilirubin 3.5mg/dL and direct bilirubin 2.4mg/dL. Abdominal CT scan showed multiple coalescing hypoattenuated peripherally enhancing masses throughout the right hepatic lobe, superior mesenteric vein thrombus, and a conglomeration of multiple nodular masses in the right lower quadrant. The patient was started on intravenous antibiotics and heparin; however, he remained febrile and symptomatic. He subsequently underwent percutaneous drainage of the hepatic abscesses. Despite these interventions, the patient deteriorated clinically. An abdominal MRI showed 10 T2 bright lesions in the right hepatic lobe and an involuting pericecal lesion. The patient underwent exploratory laparotomy, where a perforated appendix was discovered and resected. Following surgery, the patient's symptoms improved with no further signs of sepsis. He was treated with an extended course of antibiotics on discharge. Pyogenic liver abscesses (PLA) are rare and potentially life threatening infections. While most commonly caused by bacteria originating in biliary and portal tracts, perforated appendicitis has rarely been implicated. This case illustrates not only a rare etiology of PLA, but an unusual course of these two clinical entities. Literature indicates that patients classically present with appendicitis, undergo appendectomy, but continue to deteriorate until PLA are identified and treated. The example in this vignette suggests that physicians should be aware of perforated appendicitis as a cause for PLA in a patient not improving with antibiotics and drainage. Further, in cases where imaging studies are non-diagnostic, exploratory laparotomy should be considered for diagnostic and therapeutic implications.Figure 1

Multinodular bilateral breast lesions diagnosed as primary breast lymphoma in a young lactating woman

Primary breast lymphoma (PBL) is a rare neoplasm that constitutes 0.4% of malignant breast lesions and 2% of extranodal lymphomas. It is seldom distinguished preoperatively from other common forms of breast cancer. Here, we report a case of a 30-year-old woman (para 3, gravida 0) who presented with multiple bilateral nodular swellings of the breast for 2-months duration, followed by bilateral axillary lymphadenopathy after 15 days, along with low-grade fever, and generalized weakness. On examination, she was found to have multiple firm nodular mass involving both the right and the left breast, along with multiple, bilateral, matted, axillary lymph nodes. A clinical diagnosis of bilateral breast carcinoma with axillary metastasis was made. Fine needle aspiration cytology of both the breast and axillary lymph node revealed monomorphic population of scattered lymphoid cells with moderate anisonucleosis and one to two prominent nucleoli. A cytological diagnosis of PBL was made, which was subsequently confirmed with histopathology and immunohistochemistry for leukocyte common antigen and CD20. Because PBLs are uncommon malignant lesions and they usually do not have characteristic clinical and imaging findings, fine needle aspiration cytology proves to be a simple, rapid, reliable, and cost-effective procedure for successful diagnosis of PBL.

A Case of Bovine Extraskeletal Osteosarcoma Characterized by Multiple Nodular Mass in the Abdominal Cavity

臨床的に腸間膜脂肪壊死と診断された黒毛和種牛（16歳8カ月齢，雌）がと畜場に搬入された．解体時，肝臓，脾臓の漿膜面，大網，横隔膜，腹壁及び肺実質に骨様の硬組織形成を伴う多数の白色腫瘤が観察された．肉眼的に既存の骨組織に腫瘤形成は認められなかった．病理組織学的には，いずれの腫瘤も類骨性骨梁及び骨芽細胞様の腫瘍細胞から構成されていた．腫瘍細胞の異型性は低く，核分裂像もまれであったが，肺への転移が認められた．免疫組織化学的には，腫瘍細胞はosteopontin，ケラタン硫酸，S-100及びvimentinに陽性を示した．以上の結果から，本症例は牛で比較的報告の少ない骨外性骨肉腫と診断された．

Juvenile Hyaline Fibromatosis: A Rare Case with Recurrence

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease. Less than 70 cases of JHF have been reported worldwide and extremely few from India. We present a case of 4-year-old girl who presented with multiple painless nodular masses on the scalp. On fine needle aspiration cytology, a diagnosis of benign spindle cell lesion was provided, the scalp lesions were excised and on histopathology the diagnosis of JHF was made. Retrospectively, the cytology slides were reviewed and the features were consistent with the diagnosis. A diagnosis of JHF was made based on the classical clinical features, Fine Needle Aspiration Cytology (FNAC) findings and the characteristic histopathological features along with a supportive evidence based on special stains. However, the patient had a recurrence within two years. Hence, we present this rare case with recurrence.

A Case of Multisystem Endometriosis

Catamenial pneumothorax is a rare complication secondary to pleural endometriosis. We present a case of a 37-year-old-female with a history of recurrent pneumothoraces with an associated temporal relationship to the onset of her menses. In addition to her recurrent pneumothoraces, on further evaluation, she was found to have multiple nodular masses within the omentum. A thoracoscopic biopsy was subsequently performed, which showed endometrial implants within the pleural space and within the omental cavity. The radiological features and pathogenesis of this rare disease are reviewed and discussed with reference to relevant literature.

Diagnostic Exercise

A 1.4-year-old virgin female brown-hooded fancy rat presented for abdominal distention, jaundice, and dyspnea. At physical examination, a firm mass was palpable in the caudoventral abdomen as well as multiple small nodular masses associated with the abdominal viscera. At necropsy, in addition to a large mass replacing the left ovary and myriad nodules studding the peritoneal surface, there was 31 ml of abdominal effusion. By cytology, the abdominal fluid contained numerous pleomorphic vacuolated tumor cells surrounding globular pale eosinophilic to amphophilic acellular material that was strongly periodic acid-Schiff positive. Histologically, the tumor was biphasic with abundant acellular hyaline matrix that was also periodic acid-Schiff positive.

Kidney involvement by IgG4-related sclerosing disease.

A 54-year-old Caucasian woman was admitted due to persistent febrile syndrome, sweating and abdominal pain. Fever marked the onset of the patient's medical history 10 years earlier. The physical examination revealed normal findings. Laboratory data showed normal white blood cell count, mild microcytic anaemia with a normal reticulocyte count, important inflammatory syndrome (C-reactive protein 40 mg/L, IL-6 23 pg/mL), normal renal function and no urinary abnormalities. Autoimmune serology was negative. An abdominal computed tomography scan revealed multiple nodular masses in the right kidney associated with a large lumboaortic adenopathic mass (Figure 1). A renal biopsy showed severe interstitial fibrosis and tubular atrophy. The borders of the sclerotic lesion were heavily infiltrated by lymphocytes and plasma cells (Figure 2). The morphology and anatomic distribution of the lesions raised the differential diagnosis of tubulointerstitial nephritis versus a lymphoma or plasma cell dyscrasia (Figure 3). Immunohistochemistry and in situ hybridization studies (including CD3, CD20, CD68, CD138, lambda LC, kappa LC, PAX5, desmin, ALK-1, AE1/AE3 cytokeratin, HMB-45) did not support a lymphoproliferative process. CD20 and PAX5 highlighted well-formed follicles and occasional germinal centres, while CD3 stained primarily small, mature-appearing T-cells. CD138 + plasma cells were abundant and kappa/lambda ISH showed polytypic distribution. Reed-Sternberg and/or Hodgkin cells were not identified, although focally, eosinophils were prominent. However, IgG4 immunostaining showed a marked increase in IgG4-positive plasma cells at the borders of the sclerotic lesions. We made a diagnosis of IgG4-related sclerosing autoimmune disease. Evolution was favourable following immunosuppressive therapy (prednisone and cyclophosphamide), with an important decrease in the size of the lumboaortic adenophatic mass and right kidney nodular masses, and remission of fever and of the inflammatory syndrome. Fig. 1. Computed tomography scan of the abdomen: nodular masses in the right kidney associated with a large lumboaortic adenopathic mass. Fig. 2. Renal biopsy: a fibroinflammatory process extensively replaces normal renal interstitium. The borders of the sclerotic lesion are heavily invaded by a dense lymphoplasmacytic infiltrate. (haematoxylin–eosin stain; original magnification, ×20) ... Fig. 3. (A) Normal kidney architecture (right) is replaced by severe inflammatory cell infiltration and fibrosis (left). (B) Well-formed follicles are visible in the inflammatory cell infiltration (high magnification of the red square marked area in A). (C) The ... IgG4-related disease is a systemic immune-mediated disease that typically manifests as fibroinflammatory masses that can affect almost any organ system. Frequently, imaging studies of the kidney involvement by IgG4-related sclerosing disease reveals singular or multiple nodular masses affecting one or both kidneys, sometimes leading to nephrectomy to exclude renal malignancy. Moreover, histopathological diagnosis is challenging because differential diagnosis with lymphoma or plasma cell dyscrasia is difficult.

Endoscopic appearance of AIDS-related gastrointestinal lymphoma with c-MYCrearrangements: Case report and literature review

Acquired immune deficiency syndrome (AIDS)-related lymphoma (ARL) remains the main cause of AIDS-related deaths in the highly active anti-retroviral therapy (HAART) era. Recently, rearrangement of MYC is associated with poor prognosis in patients with diffuse large B-cell lymphoma. Here, we report a rare case of gastrointestinal (GI)-ARL with MYC rearrangements and coinfected with Epstein-Barr virus (EBV) infection presenting with various endoscopic findings. A 38-year-old homosexual man who presented with anemia and was diagnosed with an human immunodeficiency virus infection for the first time. GI endoscopy revealed multiple dish-like lesions, ulcerations, bloody spots, nodular masses with active bleeding in the stomach, erythematous flat lesions in the duodenum, and multiple nodular masses in the colon and rectum. Magnified endoscopy with narrow band imaging showed a honeycomb-like pattern without irregular microvessels in the dish-like lesions of the stomach. Biopsy specimens from the stomach, duodenum, colon, and rectum revealed diffuse large B-cell lymphoma concomitant with EBV infection that was detected by high tissue EBV-polymerase chain reaction levels and Epstein-Barr virus small RNAs in situ hybridization. Fluorescence in situ hybridization analysis revealed a fusion between the immunoglobulin heavy chain (IgH) and c-MYC genes, but not between the IgH and BCL2 loci. After 1-mo of treatment with HAART and R-CHOP, endoscopic appearance improved remarkably, and the histological features of the biopsy specimens revealed no evidence of lymphoma. However, he died from multiple organ failure on the 139(th) day after diagnosis. The cause of his poor outcome may be related to MYC rearrangement. The GI tract involvement in ARL is rarely reported, and its endoscopic findings are various and may be different from those in non-AIDS GI lymphoma; thus, we also conducted a literature review of GI-ARL cases.