Loredana De Marco

Aggressive angiomyxoma of the spermatic cord. Two unusual cases occurring in childhood.

We report on two cases of aggressive angiomyxoma (AAM) of the spermatic cord occurring in two 13-year-old children. Clinically, the tumor simulated a mass of the spermatic cord. Histologically, it represented a poorly circumscribed, benign myxoid tumor, with a sparse population of stromal cells immunoreactive for vimentin and, focally, for smooth muscle actin. No immunostaining for desmin, S-100, p53, p21waf-1, c-Erb-B2 and estrogen-progesterone receptors was found. High proliferating cell nuclear antigen (PCNA) immunoexpression found in most of the tumor cells may explain the high risk of recurrence. AAM should be considered in the differential diagnosis of a spermatic cord mass occurring during infancy.

Primary histiocytic sarcoma presenting as diffuse leptomeningeal disease: Case description and review of the literature

Histiocytic sarcoma is a rare malignant neoplasm arising most commonly in lymph nodes, intestinal tract, skin and soft tissue. The incidence of primary CNS histiocytic sarcoma is even rarer with a total of just 27 cases reported in the literature so far. Herein we describe the first autopsy case of histiocytic sarcoma presenting as a diffuse leptomeningeal disease in absence of a CNS tumor‐forming parenchymal lesion. The clinical, pathological and immunophenotypic features are described and an updated literature review on primary CNS histiocytic sarcoma is included.

An Italian case of intestinal anisakiasis with a presurgical diagnosis: Could this parasite represent an emerging disease?

Anisakiasis is a parasitic infection caused by the consumption of raw fish containing larvae of the Anisakis species. Since the first description in 1960 of a patient suffering from this pathogen, in the Netherlands, most of the cases have been reported in Japan, where consumption of raw fish is common, but the number of cases is increasing worldwide. The first case identified in Italy dates back to 1996 and a few cases have been reported since then. In Italy the intestinal form occurs almost as frequently as the gastric form, which is far more frequent in Japan. Intestinal Anisakiasis represents a diagnostic challenge as it is clinically misdiagnosed and most of the patients require surgery due to the occurrence of complications such as bowel occlusion or perforation. Practically no cases of the intestinal form are diagnosed preoperatively. We report the first case, to our knowledge, of intestinal Anisakiasis in which surgery was avoided, due to a prompt diagnosis suspected on intestinal biopsies. A literature review of Anisakiasis cases reported in Italy is also carried out.

Endoscopic submucosal dissection of an unusual flat rectal neoplasm

A 63-year-old woman was admitted to our gastroenterology unit to undergo a screening colonoscopy, scheduled for faecal occult blood test positivity. During the procedure, a flat, rectal granular lesion was found. With the use of magnification and narrow band image (NBI) technology, the lesion was diagnosed as a laterally spreading tumour (LST), granular mixed type, 30 mm in maximum diameter (figure 1). The pit pattern was unclassifiable and vascular pattern was similar to the type IV of the classification proposed by Inoue regarding the microvasculature pattern of the oesophagus for diagnosis and evaluation of the squamous cell carcinoma. The …

Primary classic Hodgkin lymphoma of the ileum and Epstein-Barr virus mucocutaneous ulcer of the colon: two entities compared

Primary classic Hodgkin lymphoma of the gastrointestinal tract represents a rare occurrence. A full patient’s work-up is essential in order to exclude a secondary intestinal involvement. Histologically Epstein-Barr virus mucocutaneous ulcer closely resembles Hodgkin lymphoma. The differential diagnosis between these two entities is relevant, since both the therapeutic approach and the clinical behavior are different. Herein, we describe a case of primary classic Hodgkin lymphoma arising in the ileum and a case of Epstein-Barr virus mucocutaneous ulcer of the colon, focusing on the main clinicopathological differences.

Diffuse Alveolar-Septal Amyloidosis Associated With Multiple Myeloma

A 61-year-old woman presented with a recent history of progressive worsening dyspnea and dry cough without fever. Leucocyte, platelet counts, and hemoglobin level were within normal limits. Serum protein electrophoresis revealed the presence of IgG (kappa) monoclonal protein. Bone marrow biopsy findings were consistent with multiple myeloma with 15% plasma cells showing kappa light chain restriction. A thoracic computed tomography scan (Figure 1) disclosed septal thickening, ground glass attenuations, and minute centrolobular opacities, mainly located in the lower lobes. A wedge left lower lobe pulmonary resection was performed. Histology revealed interstitial and perivascular homogeneous densely eosinophilic material (Figure 2A) associated with plasma cellular infiltrate (Figure 2B) showing kappa light chain restriction (Figure 2C). The eosinophilic deposits were orange-red with Congo red stain and displayed apple-green birefringence under polarized light (Figure 2D). The final diagnosis was diffuse alveolar-septal amyloidosis in a patient with multiple myeloma. A bortezomib-based chemotherapy was started, followed by different chemotherapeutic regimens including cyclophosphamide, thalidomide, dexamethasone, and melphalan. The patient developed progressive respiratory distress and died 7 years after diagnosis. Amyloidosis comprises different diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of different tissues and organs. Amyloidosis can be hereditary or acquired, localized or systemic. According to the World Health Organization classification, amyloidoses are defined by the different fibrillary proteins. In amyloidoses associated with an underlying plasma cell dyscrasia including multiple myeloma, the deposits are made up of immunoglobulin light chain (AL amyloidosis) or heavy chain (AH amyloidosis), with AL being the most common form. In AA amyloidosis there is tissue deposition of serum amyloid A, an α-globulin produced by the liver in different chronic inflammatory conditions. ATTR amyloidoses refer to the deposition of wild-type or mutated transthyretin in tissues, often the myocardium, in elderly patients. Three main histologic presentations of amyloidosis can occur in the lung: nodular pulmonary amyloidosis, tracheobronchial amyloidosis, and diffuse alveolar-septal amyloidosis, also known as diffuse parenchymal amyloidosis. Whereas nodular and tracheobronchial amyloidosis are 742200 IJSXXX10.1177/1066896917742200International Journal of Surgical PathologyZanelli et al research-article2017

Erdheim-Chester disease: Description of two illustrative cases involving the lung

Erdheim–Chester disease represents a clonal systemic proliferation of histiocytes. Bone is the most common site of involvement, although almost any organ, including the lungs, can be affected.

Multiple Myeloma With Multilobated Plasma Cells: An Unusual and Challenging Morphological Variant

A 70-year-old man presented with fatigue and severe back pain. He had a diagnosis of serum monoclonal gammopathy (IgG/kappa) that remained stable and asymptomatic for 7 years. On admission, the laboratory findings showed anemia with low hemoglobin level (9.5 g/dL), thrombocytopenia (31000/mmc), increased creatinine level (4 mg/ dL) with creatinine clearance 13 mL/min, hypercalcemia (14.3 mg/dL), and a serum B2 microglobulin of 1.4 mg/dL (normal value 0.10-0.25). Serum electrophoresis revealed a peak in the γ-globulin fraction, and immunofixation detected monoclonal IgG/kappa in serum (10.6 g/L) and kappa light chain in urine (3.28 mg/24 h). Bence Jones protein was present. Whole body radiographic scan and magnetic resonance imaging identified multiple lytic bone lesions in the cervical and thoracic spine and in the hip. Bone marrow biopsy showed a diffuse and massive infiltration by atypical, large multilobated and multinucleated cells with abundant cytoplasm (Figure 1), involving about 60% of bone marrow volume. The multilobated cells stained positive for CD138 (Figure 2A), MUM1/IRF4 (Figure 2B), and kappa chain (Figure 2C) and negative for CD20. A diagnosis of multiple myeloma with multilobated nuclei, stage III according Bartl, was made. Based on the cytogenetic abnormalities identified such as t(4;14), deletion of TP53/17p11, and gain (1q21), the myeloma was considered at high cytogenetic risk. After radiotherapy on bone lesions (total dose 25 Gy), the patient is currently on thalidomide, bortezomib, and dexametasone induction therapy for autologous stem cell transplantation. Multiple myeloma with multilobated/multinucleated plasma cells is a very uncommon (2% to 8%) morphological variant often characterized by an aggressive clinical course. The unusual morphological features of large bizarre multilobated plasma cells can make its diagnosis difficult to achieve. Immunohistochemistry is essential in order to avoid misdiagnosis especially with myeloid/ monocytic leukemia or nonhematological malignancies.

Chemical gastritis and colitis related to hydrogen peroxide mouthwash.

Hydrogen peroxide is a commonly used oxidizing agent with different uses depending on its concentration. Three percent solutions are used as common household disinfectants for superficial wounds. Hydrogen peroxide has been utilized widely throughout medical history. Clinical applications involving the gastrointestinal tract have included rectal enema to relieve faecal impactions in adults or meconium ileus in newborns. Nowadays most of these practices have been abandoned due to severe intestinal iatrogenic injuries [3]. Ingestion of 3% hydrogen peroxide is usually considered to be relatively nontoxic, producing only minimal gastrointestinal irritation [4]. We report a case of chemical gastritis and colitis after use of mouthwash with 3% hydrogen peroxide solution. Patient consent was obtained. A 54-year-old woman presented to our institution complaining of acute epigastric and abdominal pain associated with postprandial, nonbloody diarrhoea. She also suffered from mild pharyngalgia and a sore mouth. The symptoms had lasted for about 2 months becoming progressively worse. The patient was afebrile. Her past medical history was unremarkable. She denied taking any medication. Physical examination revealed a mild diffuse abdominal tenderness. Her blood examinations were within normal limits. Upper and lower gastrointestinal endoscopy did not reveal any abnormalities. Multiple gastric biopsies showed mild gastritis with focal superficial erosions. Microscopic examination of multiple colorectal biopsies revealed mucosal oedema, vascular congestion and mild gland atrophy. An increase in apoptotic bodies in the cryptal epithelium of the colorectal mucosa was also noted. The pathological features appeared consistent with mild ischemic colorectal damage. However, the presence of apoptosis in the crypt epithelium raised the possibility of a drug effect [5]. A detailed clinical history disclosed that the patient was having an endodontic root canal procedure to treat an infected tooth when the gastrointestinal symptoms had started. The endodontic treatment was performed once a week for 2 months. During each session 50 ml of a 3% hydrogen peroxide solution was applied to clean out and disinfect the root canal. Isolation devices to prevent the patient from swallowing the product, such as dental dams, were not used and presumably a large amount of the hydrogen peroxide solution was ingested. The gastrointestinal symptoms had started approximately 4 weeks after the beginning of the endodontic procedure and had become progressively worse during the 2-month treatment period. After suspension of the mouthwash the patient achieved a complete resolution of gastrointestinal symptoms. The history of hydrogen peroxide toxicity in the gastrointestinal tract dates back to its use with enema causing severe colonic damage similar to that of ulcerative colitis or pseudomembranous colitis [6]. Cases of chemical colitis have also been reported to occur as a result of accidental British Journal of Clinical Pharmacology Br J Clin Pharmacol (2017) 83 427–428 427

Malakoplakia Associated With Multiple Adenomas of the Colon: An Extremely Rare Incidental Finding.

Malakoplakia in the colon has been described either as part of a systemic disease with a diffuse presentation or as a rare incidental finding associated usually with an adenocarcinoma. To our knowledge, the association of malakoplakia with colonic adenoma is very rare and only 5 cases, including the present case, have been reported so far. Here, we describe a case of malakoplakia coexisting with multiple sigmoid colonic adenomas in a 67-year-old patient without any underlying inflammatory or infectious disease and with no history of immunosuppressive treatment. As far as we are concerned, this is the first description of malakoplakia associated with multiple colonic adenomas.