An Update on Myocarditis in Forensic Pathology.

In forensic medicine, myocarditis is a complicated topic in the context of sudden death and medical malpractice. A good knowledge of the etiopathology, histopathology, and available literature are both indispensable and essential for the correct management and evaluation of the causal link. Some agents, which are rarely lethal for humans, are not necessarily related to death from myocarditis, even if an infection in other organs such as the gastrointestinal tract is documented. The diagnosis of the causes of death is often difficult and confusing. In some cases, the hypothetical diagnosis of myocarditis as the cause of death is formulated by deduction, causing error and misleading the correct temporal evaluation of pathological events. We reviewed the literature realizing that histomorphological data are scarce and often poorly documented. Only after COVID-19 have the histomorphological aspects of myocarditis been better documented. This is due to poor autopsy practice and poor accuracy in identifying the specific histotype of myocarditis with identification of the responsible agent. We believe that four points are essential for a better understanding and complete diagnosis of the disease: (1) clinical classification of myocarditis; (2) etiological classification of myocarditis; (3) pathophysiology of viral and bacterial infections with host response; and (4) histopathological diagnosis with precise identification of the histotype and pathogen. In the review we provide histological images from authoritative scientific references with the aim of providing useful information and food for thought to readers.

Central and Peripheral Nervous System Complications of Vasculitis Syndromes from Pathology to Bedside: Part 2-Peripheral Nervous System.

PURPOSE OF REVIEW: Peripheral nervous system vasculitides (PNSV) are a heterogeneous group of disorders with a clinical subset that may differ in prognosis and therapy. We provide a comprehensive update on the clinical assessment, diagnosis, complications, treatment, and follow-up of PNSV. RECENT FINDINGS: Progress in neuroimaging, molecular testing, and peripheral nerve biopsy has improved clinical assessment and decision-making of PNSV, also providing novel insights on how to prevent misdiagnosis and increase diagnostic certainty. Advances in imaging techniques, allowing to clearly display the vessel walls, have also enhanced the possibility to differentiate inflammatory from non-inflammatory vascular lesions, while recent histopathology data have identified the main morphological criteria for more accurate diagnosis and differential diagnoses. Overall, the identification of peculiar morphological findings tends to improve diagnostic accuracy by defining a clearer boundary between systemic and non-systemic neuropathies. Therefore, the definition of epineurium vessel wall damage, type of vascular lesion, characterization of lymphocyte populations, antibodies, and inflammatory factors, as well as the identification of direct nerve damage or degeneration, are the common goals for pathologists and clinicians, who will both benefit for data integration and findings translation. Nevertheless, to date, treatment is still largely empiric and, in some cases, unsatisfactory, thus often precluding precise prognostic prediction. In this context, new diagnostic techniques and multidisciplinary management will be essential in the proper diagnosis and prompt management of PNSV, as highlighted in the present review. Thirty to fifty percent of all patients with vasculitis have signs of polyneuropathy. Neuropathies associated with systemic vasculitis are best managed according to the guidelines of the underlying disease because appropriate workup and initiation of treatment can reduce morbidity. Steroids, or in severe or progressive cases, cyclophosphamide pulse therapy is the standard therapy in non-systemic vasculitic neuropathies. Some patients need long-term immunosuppression. The use of novel technologies for high-throughput genotyping will permit to determine the genetic influence of related phenotypes in patients with PNSV.

A case report of HCC cutaneous metastasis.

INTRODUCTION: Hepatocellular carcinoma (HCC) is one of the most common solid tumors in the world. HCC muscle and cutaneous metastases are rare and can occur by hematogenous spread or following surgical procedures performed for diagnostic or therapeutic aims. CASE STUDY: We present a 47-year-old man underwent liver biopsy followed by surgical resection for moderately differentiated HCC in January 1999. In February 2010 the patient presented with HCC cutaneous metastases in the surgical scare and with HCC metastases of the right abdominal rectus muscle. En bloc tumor resection with the right abdominal rectus muscle was performed. In March 2011 the patient underwent resection of a locoregional muscle relapse. In November 2011 we started Sorafenib for intraepatic recurrence. DISCUSSION: This case suggests that in the follow-up of patients who underwent surgical resection or diagnostic and therapeutic procedures for HCC should be considered the possibility of abdominal wall metastasis even after many years, particularly in the cases with favorable evolution.

Acinic cell carcinoma of the breast arising in microglandular adenosis.

Acinic cell carcinoma is a rare breast tumour belonging to salivary gland-like tumours of the breast. They are "triple-negative" breast cancers even if their biological behaviour seems to be more favourable. Herein we present an acinic cell carcinoma arising on a background of typical and atypical microglandular adenosis in a 58-year-old woman, along with a review of the literature.

Gastric duplication cyst: a rare congenital disease often misdiagnosed in adults.

Gastrointestinal duplication is a rare congenital disease which affected more commonly the ileum, while the stomach is rarely involved. Generally diagnosed in paediatric or young age, it could be difficult to suspect a gastrointestinal duplication in adults. Herein, we report a 55-year-old male with a gastric duplication cyst found on routinely checkup for chronic hepatitis and first misdiagnosed as a gastrointestinal stromal tumor (GIST); we also discuss its embryology.

Cystic variant of endometrial stromal sarcoma: report of two cases.

Endometrial stromal sarcoma is an uncommon tumor representing 0.2% of all uterine neoplasm and 15% to 26% of uterine sarcomas. Endometrial stromal sarcoma has a gross appearance as single nodule, multiple masses, or a poorly demarcated lesion with occasional cystic degeneration; rarely, it shows a cystic multilocular feature. We report 2 cases of endometrial stromal sarcoma with a prominent cystic appearance, forming multilocular cystic mass, detected by ultrasonography. The differential diagnosis among multicystic endometrial stromal sarcoma and cystic uterine tumors might be very difficult.

Mast cell sarcoma of the scalp: the first sign of undisclosed systemic mastocytosis?

Mastocytosis is a neoplastic disease of mast cells and their CD34+ precursors, including a heterogeneous group of disorders. It is characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Mast cell sarcoma is an extremely rare and aggressive disease characterized by local proliferation of atypical mast cells, destructive growth and poor prognosis, without systemic involvement. Very few clinical cases describing this entity have been reported in the literature. In this paper, we report a case of a mast cell sarcoma, localized in the scalp of a 63-year-old woman; it appears to be the first manifestation of undisclosed systemic mastocytosis.

Symplastic glomus tumor: report of a challenging lesion with literature review.

Glomus tumors are uncommon mesenchymal tumors whose cells closely resemble those of a normal glomus body, and are found most commonly in the hand. Recently, a symplastic form exhibiting marked nuclear atypia, in the absence of any other malignant features, has been described. To date, only 14 cases of symplastic glomus tumor have been reported in the literature; hence, very little information is available about its diagnosis, treatment, and biological behavior. The case reported here concerns a symplastic glomus tumor occurring in the right index finger of a 62-year-old woman. We reviewed the literature concerning previously reported cases, placing emphasis on the documented biological behavior, treatment, and demographic trend. Physicians must be aware of this morphological variation of glomus tumor to avoid the mistake of malignancy, which results in over-treatment of the patient.

Cutaneous needle track seeding of mesothelioma diagnosed by fine needle aspiration cytology: a case report.

BACKGROUND: Diagnostic and therapeutic procedures for mesothelioma require surgical biopsy or the usage of different-sized needles. Thoracic wall involvement along the surgical or needle tracks has been reported. CASE: A 57-year-old woman who had suffered from non-Hodgkin's lymphoma complained of dyspnea and left pleural effusion. The patient had been treated with chemotherapy and radiotherapy and was in remission since then. Thoracentesis was performed using a 22-gauge needle; the cytologic diagnosis was malignant pleural mesothelioma. Within 2 weeks from thoracentesis, the patient complained of an erythematous swelling in her left chest wall, in the area of the needle track. Fine needle aspiration cytology (FNAC) of the swelling was performed using a 23-gauge needle; 2 smears and a cell block were prepared. Smears showed neoplastic cells in sheets and papillary configuration with the features of mesothelial lineage. Immunocytochemistry showed positivity for calretinin and vimentin. Cytologic slides of the former effusion showed an overlapping of the cytologic and immunocytochemical features. A diagnosis of chest wall involvement from mesothelioma was established and histologically confirmed. CONCLUSION: Chest wall infiltration is a definite risk in the management of pleural mesothelioma, and FNAC is a useful procedure for a timely diagnosis of this ominous complication.

Diprosopus conjoined twins: radiologic, autoptic, and histologic study of a case.

Conjoined twins are a rare and intriguing nature's phenomena; diprosopus or craniofacial duplication is the rarest with a reported incidence of 1 case in 180,000-15 million births. We present a radiologic, autoptic, and histologic study of a 37-week-old male diprosopus twin in a dichorionic pregnancy of a 26-old-year woman. Diprosopus malformation is part of duplication involving face and cranium like janiceps and dicephalus. Our case also shows partial duplication of the stomach with ectopic pancreas. Most studies are required to understand the exact mechanism of this malformation.

Angiosarcoma mimicking rhinophyma.

We report the case of a 61-year-old man showing persistent erythematous macules, plaques, and partially confluent nodules with irregular borders, developed on his nose for one year. During that time the patient underwent several dermatological consultations, and all produced the same diagnosis: rhinophyma. So antibiotic and steroid treatment was carried out without any improvement while the lesions kept growing. When the patient came to our observation, physical examination revealed large, infiltrative, oedematous, erythematous plaques and rare nodules, with superficial telangiectatic vessels. Cervical lymphadenopathy was not detectable. Routine laboratory analysis was normal. Punch biopsy was performed, and histopathology and immunohistochemical studies were consistent with cutaneous angiosarcoma. This is the report of a face angiosarcoma with an unusual and very deceptive clinical presentation.

Unexpected autoptic finding in a sudden death: gossypiboma.

Gossypiboma, i.e. a retained surgical sponge, is a serious and rare complication in surgical practice, most commonly occurring in abdominal procedures. Migration of retained surgical sponge is an unusual sequelae, particularly if occurring in tracheo-bronchial tree. Herein, we report a 35-year-old man who had had a retained surgical sponge forgotten during a radical thyroidectomy, and subsequently a trans-luminal migration of the gossypiboma which went through the trachea causing a sudden death of the patient. All the operators of the surgical team should keep in mind this terrible complication to avoid unpleasant consequences to patient and themselves.

Endocrine carcinoma of the major papilla: report of two cases and review of the literature.

To date, about 100 cases of ampullary NET are reported in International literature. These tumors can cause symptoms mainly secondary to their periampullary location. Up to 25% of patients have von Recklinghausen's disease. Carcinoid syndrome is uncommon, unless hepatic metastasis is present. Determination of histopathology is of utmost importance and involves specific immunohistochemical staining. The published data indicate that these tumors, metastasize in approximately half of cases irrespective of primary tumor size. Therefore, radical excision in the form of pancreaticoduodenectomy is recommended regardless of tumor size. Local excision should be confined to patients unable to tolerate more extensive surgery. We here report two case of ampullary neuroendocrine tumors presenting as melena and painless jaundice respectively in a 51-year old man and in a 54-year old man and review the relevant literature, giving special attention to the morphologic features, clinical characteristics, and treatment modalities associated with this disease process.

A case of pure uterine lipoma: immunohistochemical and ultrastructural focus.

BACKGROUND: Pure uterine lipoma is a rare clinical event and only a few cases have been reported in literature. The histogenesis of these lesions is still debatable. Preoperative diagnosis is difficult and should be pathologically confirmed postoperatively. CASE: We report the case of a 58-year-old woman who presented with pelvic pain and postmenopausal uterine bleeding. The hysterectomy specimen showed a pure intramural lipoma of the uterus. An immunohistochemical study revealed that the lipomatous tissue was reactive to S-100, vimentin, actin and desmin. Electron microscopy examination revealed bundles of spindle cells with intracytoplasmatic vacuoles and parallel-arranged intermediate filaments in the surrounding zone, in which adipose cells were mixed with muscular cells. DISCUSSION: Clinical and histological diagnosis of pure uterine lipomas are described and a possible involvement of fatty metaplasia of smooth muscle cells in the development of pure uterine lipomas is discussed.

Nodal and extranodal soft tissue polymorphous hemangioendothelioma: a case report and review of the literature.

BACKGROUND: Polymorphous hemangioendothelioma is a rare vascular tumor of borderline malignant potential and only 10 cases have been described in the literature so far. METHODS AND RESULTS: We report a case of nodal and extranodal polymorphous hemangioendothelioma and review the literature. A 66-year-old man with an unremarkable past medical history was admitted for a left submandibular mass. The main nodule was composed of angiosarcoma-like areas mixed with angiomatous features; some vascular spaces with hobnail endothelium were seen. The tumor involved two adjacent lymph nodes. Immunohistochemically, the neoplastic cells were strongly positive for CD31 and vimentin. After a few months the tumor recurred and the patient was treated with radiation therapy. CONCLUSIONS: Polymorphous hemangioendothelioma is a distinct entity in the hemangioendothelioma group with its own clinical and histological features.

Analysis of Cox-2 expression in Wilms' tumor.

Cylooxygenase-2 (Cox-2) inhibitors have increasingly become therapeutic alternatives in some Cox-2-overexpressing neoplasms. As the treatment eligibility for these drugs hinges on Cox-2 expression, Cox-2 immunostaining has recently been widely examined in several malignant neoplasms. However, data on the expression of Cox-2 in Wilms' tumor (WT) are limited. In this study, we examined Cox-2 expression in 40 examples of WT to identify the prognostic impact, to evaluate the effects on tumorigenesis, and to answer the question of whether neoplasms with Cox-2 expression could benefit from treatment with specific Cox-2 inhibitors. Sections from paraffin-embedded tumor samples were immunostained by a standard ABC technique using Cox-2 mouse monoclonal antibody. As in other rare examples reported in the literature, Cox-2 immunoreactivity was analyzed and correlated with histological features and the staging of neoplasms. However, in contrast to other studies, we also evaluated the relation of Cox-2 positivity to age, sex, and survival of patients. The results of this study demonstrated that Cox-2 was ubiquitously expressed in all cases of WT and their neovasculature, independently of the type of neoplasm (tumors with a favorable or unfavorable histology), tissues which constitute the neoplasm (blastemal, mesenchymal and epithelial, heterologous or non-heterologous elements), patient age, sex, or stage of development and survival rate. Thus, Cox-2 inhibitors could be used for treating all cases of WT. Further studies, including molecular investigations, would be useful to confirm our hypotheses.

Basal cell carcinoma with fibroepithelioma-like histology in a healthy child: report and review of the literature.

We report the case of a 13-year-old girl with a 7 mm plaque on the trunk. The mother of the patient reported that the lesion developed at the age of 3 and it was characterized by a slow growth. In 2003 a dermoscopic examination was executed and a diagnosis of dermical nevus was made. On examination at our department, a red-pinkish plaque with two small areas of ulceration was found on the anterior part of the trunk. At dermoscopic examination we observed vascular pattern with linear-irregular vessels, multiple blue-gray dots, blue-gray ovoid nests, and ulceration. A diagnosis of basal cell carcinoma was made and confirmed by histopathologic examination. The histopathologic specimen showed nests or lobules of basalioid cells associated with a surrounding loose fibromucinous stroma; two patterns were observed: adenoid-cystic and fibroepithelial. Some fibroepithelioma of Pinkus-like areas were present, where the fibrovascular stroma was very abundant with an arborizing network of long, thin cords of basaliod cells that extended downward from the epidermis and created a fenestrating pattern. The lesion was surgically excised. Basal cell carcinoma occurs rarely in children and is most often associated with an underlying condition that predisposes patients to the development of malignancy that was not present in our case.