Bone Marrow-Liver-Spleen Type of Large B-Cell Lymphoma Associated with Hemophagocytic Syndrome: A Rare Aggressive Extranodal Lymphoma

Recently, an unusual subtype of large B-cell lymphoma (LBCL) with distinctive clinicopathologic features has been recognized; it is characterized by involvement of bone marrow with or without liver and/or spleen, but no lymph node or other extranodal sites, usually associated with fever, anemia, and hemophagocytic lymphohistiocytosis (HLH). Because of this distinctive clinical presentation, it has been designated “bone marrow-liver-spleen” (BLS) type of LBCL. To date there is only one series of 11 cases of BLS type of LBCL with detailed clinical, pathologic, and cytogenetic data. Herein, we describe a case of BLS type LBCL presenting with associated HLH in a 73-year-old female. The bone marrow core biopsy showed cytologically atypical large lymphoma cells present in a scattered interstitial distribution and hemophagocytosis and infrequent large lymphoma cells were seen in the bone marrow aspirate smears. Circulating lymphoma cells were not seen in the peripheral blood smears. The patient underwent treatment with chemotherapy (R-CHOP) but unfortunately passed away 2 months after initial presentation. BLS type of LBCL is a very rare and clinically aggressive lymphoma whose identification may be delayed by clinicians and hematopathologists due to its unusual clinical presentation and pathologic features

Pulmonary Empty Spaces: Silicone Embolism—A Decade of Increased Incidence and Its Histological Diagnosis

Pulmonary embolism (PE) is a critical complication related to multiple disorders and different medical or cosmetic procedures. This case report presents two patients who were admitted for respiratory symptoms in the setting of previously receiving silicone injections for cosmetic purposes and were diagnosed with silicone pulmonary embolism. The relevance of including questions about all cosmetic procedures as a part of a medical history is highlighted, in particular about silicone injections. The diagnosis is confirmed by histological means. Additionally, our review showed the change of most common sites of silicone injections and a significant increase in cosmetic procedures causing silicone embolism during the past twelve years

Dedifferentiated Liposarcoma With Rhabdomyosarcomatous Differentiation Producing HCG: A Case Report of a Diagnostic Pitfall

We report a first case of paraneoplastic human chorionic gonadotropin (HCG) production in a dedifferentiated liposarcoma with rhabdosarcomatous differentiation in an 83-year-old man with a retroperitoneal mass, unilateral scrotal enlargement, and a serum HCG level of 843 IU/L. Core biopsy of the retroperitoneal mass revealed rhabdomyosarcoma. Orchiectomy revealed a paratesticular dedifferentiated liposarcoma with rhabdosarcomatous differentiation. Fluorescence in situ hybridization analysis performed on both the retroperitoneal and paratesticular masses revealed amplification of MDM2. The retroperitoneal tumor was interpreted as metastatic dedifferentiated liposarcoma with the dedifferentiated component represented by rhabdomyosarcoma. HCG production is a common feature of testicular germ cell tumors, less common in carcinomas, and extremely rare in sarcomas. Accordingly, sarcomas secreting HCG are a potential diagnostic pitfall, and raise the differential diagnosis of germ cell tumors and a variety of carcinomas. HCG production by carcinomas is a known poor prognostic finding, however the significance of its production in sarcomas is unknown

Hemangioma of a Male Breast: Case Report and Review of the Literature

BACKGROUND Male breast hemangioma is a rare benign neoplasm that is usually excised for definitive diagnosis. CASE REPORT In our case report, we present a male octogenarian with multiple comorbidities who presented with a large palpable mass in his right breast. The diagnostic imaging studies were suggestive of a benign tumor, with a BI-RADS (Breast Imaging Reporting and Data System) score of 3. Subsequent core needle biopsies were diagnostic of benign hemangioma. The patient was managed with observation due to his comorbidities. Benign vascular tumors in the male breast are exceptionally rare, and in our review of the literature we found only 14 previously published cases. Historically, fine needle aspiration has been found to be unreliable in making a definitive diagnosis and surgical excision has been the standard treatment. CONCLUSIONS Recent studies and our case report indicate that core needle biopsy may be a reliable diagnostic tool and observation is a possible option for hemangiomas in male patients who cannot undergo surgery

Pseudofungi: A Diagnostic Pitfall

Pseudofungi are septate hyphae-like structures very similar morphologically to true fungal elements. Histologically their presence poses a diagnostic challenge as they mimic fungal infection. Accurate identification of pseudofungi is required to avoid unnecessary treatment, and this can be accomplished by careful morphologic analysis with the use of appropriate histochemical stains. We report a patient with pseudofungi within a paratracheal lymph node, describe the morphologic findings, and discuss the differential diagnosis

Mesenteric desmoid tumour presenting with recurrent abdominal abscess and duodenal fistula: A case report and review of literature

•Mesenteric desmoid is a rare benign tumor, usually asymptomatic, but can grow aggressively and cause complications.•Recurrent intralesional abscess formation even after drainage and medical therapy should lead to the assumption of fistulization to the adjacent bowel.•Treatment should be guided by a multidisciplinary team, and operation intervention should be performed by surgeons familiar with desmoid tumor biology. Desmoid tumors are locally destructive but histologically benign. Their management involves close observation and surgical, medical, or hormonal treatment. A 36-year-old male was admitted for abdominal pain and fever. A CT scan showed fluid collections and air within a mesenteric mass. Diagnostic laparotomy was performed with drainage of the abscess and biopsy of the mass. The pathology suggested a desmoid tumor. His fever and abdominal pain persisted. An endoscopy was performed, which demonstrated a fistula track in the third part of the duodenum. After a multidisciplinary discussion, consensus was to pursue surgical intervention. The patient underwent an en bloc resection of the tumor including a portion of the wall of the third part of the duodenum. The final pathology confirmed a desmoid tumor with a fistula track to the duodenum. The patient had a re-laparotomy on POD2 for intra-abdominal bleeding but was discharged without further events on POD7. He had no evidence of recurrence on follow-up at 11 months. Desmoid tumors are rarely complicated by abscess formation or fistulization. The management of intra-abdominal desmoids in this setting is challenging, as patients are often symptomatic and unresponsive to medical management. Percutaneous drainage and antibiotics are often initiated as first-line treatment, followed by surgery or medical therapy after evaluation of resectability and tumor stage. Rare complications can arise with intra-abdominal desmoid tumors. Principles of infection control should be applied in combination with optimization of oncologic outcome. A multidisciplinary approach helps to achieve both these objectives

Classic Hodgkin lymphoma and Castleman disease: an entity appears to be emerging

Hodgkin lymphoma is a B cell neoplasm characterized by Hodgkin and Reed-Sternberg (HRS) cells in an inflammatory background. Classic Hodgkin lymphoma (CHL) accounts for approximately 90% of all cases of HL and four types are recognized in the World Health Organization (WHO) classification: lymphocyte-rich, nodular sclerosis, mixed cellularity, and lymphocyte depleted. Castleman disease (CD) is a designation used for a heterogeneous group of diseases that involve lymph nodes. Histologically, there are hyaline vascular and plasma cell variants, the latter including human herpes virus 8 (HHV8)-positive and HHV8-negative subsets. In this study, we describe three men, 45-57 years of age, one HIV-positive, who had coexistent CHL and CD. All patients had the interfollicular variant of CHL and HHV8-negative plasma cell variant CD. Immunohistochemical analysis supported the diagnosis of CHL; the HRS cells were positive for CD15, CD30, and PAX-5 (dim). In two cases, the HRS cells and the plasma cells of CD expressed interleukin-6 (IL-6). Our review of the literature identified 34 cases of coexistent CHL and CD reported previously. In aggregate, about two-thirds of all cases of CHL have been the interfollicular variant and around 90% of CD cases were plasma cell variant, HHV8-negative in the subset of cases tested. We suggest that interfollicular variant CHL and plasma cell variant CD may be a distinct entity with a common pathogenesis, possibly related to IL-6 dysregulation. The few cases in the literature describing other forms of CHL and hyaline vascular variant CD are different from the entity reported here, with a different pathogenesis, likely similar to focal Castleman-like changes that have been described in association with various types of non-Hodgkin lymphoma