A rare case of mucoepidermoid carcinoma of the skin: A case report and a literature review

This report presents the case of primary Mucoepidermoid Carcinoma of the skin on the leg of a 74-year-old man. The epidemiological data of the neoplasm are examined, and the morphological picture and immunophenotypic profile are compared with those of the homologous tumor of the salivary glands. According to the scoring system of this type of tumor, our case is classified as low-grade.

A confluence of rarities: post sedation neuroexcitation following adrenal perineurioma resection

Adrenal masses are frequently discovered incidentally and warrant further workup to explore the etiology of the mass. We present the case of a patient who had an incidentally discovered nonfunctional adrenal mass, which was determined to be a perineurioma. This is the first case report, to our knowledge of a perineurioma occurring in this location. Additionally, we report an episode of post sedation excitation, or “propofol frenzy” in the same patient to add to the clinical spectrum of presentation of this phenomenon.

Nodal mycobacterial spindle cell pseudotumor: A diagnostic pitfall

Inflammatory pseudotumor (IPT) is a rare benign mass forming lesion that has been reported in virtually every organ, and can closely mimic spindle cell neoplasms. Mycobacterial spindle cell pseudotumor (MSP) represents a small proportion of IPT of the lymph node, which occur in immunocompromised patients, posing a diagnostic challenge. We report three cases of MSP involving two AIDS patients who presented with generalized lymphadenopathy, and one immunosuppressed patient with a mediastinal mass. Biopsy in these cases revealed effaced architecture replaced by proliferating fibrohistiocytic spindle cells, fibrosis and polymorphic inflammatory infiltrate. Inflammatory pseudotumors and other spindle cell neoplasms of the lymph node can show overlapping morphologic features, resulting in diagnostic confusion. A differential diagnosis of Mycobacterial spindle cell pseudotumor should be kept in mind when approaching localized or generalized lymphadenopathy in an immunocompromised patient.

Malignant granular cell tumor of the ulnar nerve: A case report of long-term follow-up and literature review

Background: The incidence of malignant granular cell tumor, an extremely rare Schwann cell-derived tumor with a poor prognosis, is reported to be approximately 0.2% of malignant soft tissue tumors. We report a case of a malignant granular cell tumor originating from the ulnar nerve.Case presentation: A 71-year-old woman presented with a mass in her right forearm. Magnetic resonance imaging showed a tumor with homogenous intensity of T1 and heterogeneous hyperintensity of T2, continuous with the ulnar nerve. Incisional biopsy revealed a malignant granular cell tumor, and marginal excision of the tumor was performed. Histologically, the tumor size was 9.2 cm and consisted of eosinophilic, granular polygonal to round and spindle-shaped cells, with vesicular and prominent nucleoli, and increased mitosis. Immunohistochemically, the tumor cells were positive for S-100 protein, CD68, H3K27me3, TFE3, and SOX10 and negative for smooth muscle alpha-actin, desmin, cytokeratin AE1/3, epithelial membrane antigen, and synaptophysin. The Ki-67 positivity rate was 12%. These findings were consistent with those of malignant granular cell tumors. In addition, no metastasis or recurrence was observed 15 years after the excision.Conclusion: Surgical resection is the standard treatment option. In our case, the diagnostic criteria for malignant granular cell tumors were histologically met. Patients with malignant granular cell tumors have a poor prognosis. However, no metastasis or recurrence was observed in this case 15 years after the surgery.

Epithelioid angiosarcoma of the small intestine presenting with intractable gastrointestinal bleeding: An uncommon tumor with diagnostic challenge and poor prognosis

Introduction: Angiosarcoma of the gastrointestinal tract is a rare, aggressive malignant neoplasm of vascular origin. Commonly, this tumor occurs due to metastases from other sites like the head and neck region and the soft tissue. When occurring as a primary tumor of the intestines, this entity poses a diagnostic dilemma due to its non-specific clinical presentation, coupled to its infiltrative features that may occur without an obvious lesion amenable to routine gastrointestinal interventions. The findings from an autopsy and surgical case with a brief review of its diagnostic features and relevant literature are discussed.Methods: We present a 73-year-old male with a history of a recent onset of gastrointestinal bleeding who was referred to our facility for persisting bleeding. He underwent endoscopy without an identifiable source of bleeding and subsequently received a colectomy at our facility, with repeated blood transfusions for persisting gastrointestinal bleeding. He was eventually transitioned to hospice and died shortly after presentation. A complete autopsy was performed.Results: There was anasarca with hemoperitoneum and intestinal intraluminal blood. Present in the small intestinal mucosa were three hemorrhagic nodules. Histologically, the nodules demonstrated medium to large epithelioid vasoformative neoplastic cells infiltrating through the wall of the small intestine and extending to the omentum. The findings from the gross and histology examination, as well as the immunohistochemical stains were consistent with an epithelioid angiosarcoma.Conclusion: Primary epithelioid angiosarcoma of the intestine is an uncommon aggressive tumor that poses a diagnostic challenge. Although rare, this diagnosis needs to be considered in patients with intractable gastrointestinal bleeding. Due to its morphologic overlap with other benign and malignant neoplasms, the use of ancillary testing during histologic examination is key in arriving at an accurate diagnosis.

Anaplastic pilocytic astrocytoma of the cerebellum presenting with conus medullaris drop metastasis

Due to its rarity, a complete understanding of the clinical behavior, pathogenesis, and diagnostic definition of anaplastic pilocytic astrocytoma (APA) is currently lacking. The optimal clinical management and use of adjuvant therapies has yet to be defined. We present a 64 year-old-female with progressive headaches, dysarthria, and ataxia, who was found to have right cerebellar mass. A gross total resection was achieved through two staged operations. Pathology demonstrated focal areas of necrosis, tumor infiltration, and increased mitotic activity most consistent with APA. Adjuvant chemotherapy and stereotactic radiosurgery were administered. Approximately two years later, the patient presented with symptoms of cauda equina syndrome, and lumbar spine imaging demonstrated a large intradural mass at the conus medullaris with diffuse leptomeningeal enhancement. A biopsy was performed and was consistent with metastatic APA. APA may rarely progress to metastatic disease, most frequently involving the leptomeninges of the posterior fossa and cervical spine. This report represents the first case of metastases distal to the cervicomedullary junction.

Urinary bladder paraganglioma metastatic to the lung in a patient with SDHB gene mutation: A case report

Introduction: Paragangliomas are tumors originating from the neural crest. Most of them are benign and arise from various locations in the body. Extra-adrenal paragangliomas arise as sporadic cases in most settings or as part of heredofamilial syndromes in about one-quarter of cases. Succinate dehydrogenase subunit B (SDHB) gene mutations are associated with an aggressive clinical disease course of pheochromocytoma/paraganglioma.Methods: We present a 41-year-old male former smoker with a history of a growing right upper lung nodule on chest imaging. He had no cough or respiratory symptoms. Twenty-seven months prior, the patient underwent a cystoprostatectomy due to paraganglioma of the bladder. Genetic testing identified a pathogenic mutation in SDHB gene, c.166_170delCCTCA (p.Pro56Tyrfs*5). He underwent a wedge resection of the lung nodule.Results: Sectioning of the lung wedge revealed a well-circumscribed, firm tan nodule. Microscopically there were nests of large neoplastic cells with round nuclei and eosinophilic granular cytoplasm. Tumor cells were positive for synaptophysin and chromogranin and negative for pan-cytokeratin. S-100 protein highlighted sustentacular cells. Morphologically, the pulmonary neoplasm was similar to the primary tumor of the bladder. These features are consistent with a bladder paraganglioma metastatic to the lung, in a background of a hereditary paraganglioma syndrome.Conclusion: Extra-adrenal paraganglioma occurring in a setting of hereditary paraganglioma syndrome has a high risk of metastasis. Lifelong surveillance even after prompt resection of the primary tumor with negative margins is required to ensure early detection of metastasis and prevent complications associated with it.

Testicular Lymphoma manifesting as neurological symptoms due to secondary neurolymphomatosis: A case report

Neurolymphomatosis is a rare manifestation of lymphoma presenting as diffuse invasion and involvement of peripheral and spinal nerves. Due to the common presenting symptomatology of neurologic complaints localizing to the affected peripheral nerve, lymphoma as the underlying etiology can be diffificult to diagnose. Here we present the case of a gentleman presenting with right extremity neuropathic symptoms, subsequently discovered to have diffuse large B-cell lymphoma of testiticular orgin after nerve biopsy revealed neurolymphomatous involvement of a spinal nerve. This case highlights the importance of the consideration of neurolymphomatosis in the work up of neuropathic symptoms, as well as the full assessment for the site of primary involvement.

Breast tall cell carcinoma with reversed polarity with an unusual molecular profile

Breast tall cell carcinoma with reversed polarity (TCCRP) is rare and previously referred to as solid papillary carcinoma with reverse polarity. This low grade tumor commonly exhibits IDH2 p.Arg172 mutation, however is not completely understood at the molecular level. We present a case of TCCRP in a 55 year old woman with a 0.7 cm left breast mass. A core biopsy was performed with immunohistochemistry. Lumpectomy and sentinel lymph node biopsy were completed two months later. MammaPrint$^{\textregistered}$ and BluePrint$^{\textregistered}$ gene expression profilers were performed on an excision block. Microscopically, the tumor was composed of circumscribed nests of columnar cells, with focal papillary architecture. Tumor cells had apically located nuclei with grooves and rare inclusions. Tumor cells were positive for CK5, IDH1/2, and calretinin, and myoepithelial cells were absent. BluePrint$^{\textregistered}$ subtyped the tumor as basaloid. MammaPrint$^{\textregistered}$ classified the tumor as high risk for metastasis. TCCRP presents a diagnostic challenge. Although these rare breast carcinomas are generally reported to have an indolent clinical course, molecular analysis by gene expression profiling classified this tumor as high risk of recurrence with a basaloid type. Therefore, molecular analysis of this tumor may lead to conflicting data regarding prognosis and treatment considerations. Clinicians and patients should weigh published data and individual prognostic information for treatment planning. Our patient and clinical team opted for radiation without chemotherapy. More cases of TCCRP need to be studied to better understand its molecular profile.

Hepatic carcinosarcoma with rhabdomyosarcoma: A case report and review of literature

Objective To analyze the clinical and pathological manifestations of a hepatic carcinosarcoma case with rhabdomyosarcoma components (HCSR).Methods A case of HCSR was observed by macroscopy, microscopy, immunohistochemistry and electron microscopy, along with thorough review of correlated literatures.Results The tumor tends to occur in elder patients without differences on gender. Epigarstric sicknesses, AFP rising in serum, and a mass on the right liver shown in radiography are commonly initial signs. It was composed of both hepatic carcinoma and variously differentiated sarcoma components, with identifiable rhabdomyosarcoma. Immunohistochemistry showed that the hepatic carcinosarcoma was positive of epithelial markers and mesenchymatous component was diffuse positive of Vimentin, and mosaic positive of SDHB, CD117, while rhabdomyosarcoma was positive of muscular markers. Transmission electron microscopy showed the tumor had both epithelial and rhabdomyosarcoma ultra-microstructure.Conclusion HCSR is a very rare type and highly malignant tumor with a dismal prognosis, hardly demonstrating unique clinical manifestations. Diagnosis and differential diagnosis rely on combination of histomorphology, immunohistochemistry and ultra-microstructure observation.