Remission of refractory granulomatous primary cutaneous anaplastic large cell lymphoma to brentuximab vedotin.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare non-Hodgkin's lymphoma that arises as a single, or multiple dome-shaped tumours on the skin. The histology is characterised by the presence of atypical lymphocytes with large irregularly shaped nuclei that express the surface marker CD30. There can be significant heterogeneity in clinical manifestation and histological pattern and in rare cases accurate diagnosis can be a challenge. Here, we present an unusual case presentation of cutaneous CD30+ anaplastic large cell lymphoma with significant granulomatous histology pattern that mimicked sarcoid. After a lack of durable response to treatments that included glucocorticoid and methotrexate, targeted treatment with anti-CD30 monoclonal antibody drug conjugate (brentuximab vedotin) yielded long-term clinical remission.

Gastrin Staining in Inflamed Stomach Biopsies Labeled as "Antral" Rarely Detects Atrophic Gastritis.

OBJECTIVES: Autoimmune metaplastic atrophic gastritis (AMAG) is an underrecognized entity, especially in its early stage. This study assessed whether the use of gastrin immunohistochemistry would increase sensitivity for diagnosing early AMAG. METHODS: Three-hundred gastric biopsies were prospectively stained for gastrin by immunohistochemistry. Inclusion criteria included well-oriented gastric mucosa with mucus glands and minimal plasma cell infiltrate not suspected to represent pyloric metaplasia. Patient age, sex, designated location of biopsy, presence or absence of intestinal metaplasia, and clinical information were not criteria. Any case with absence of gastrin-positive endocrine cells reflexed to chromogranin immunohistochemistry. Maloriented biopsies or cases with current Helicobacter infection were excluded. RESULTS: The 298-patient study cohort comprised 222 females (mean age, 47 years; range, 16-80 years) and 76 males (mean age, 49 years; range, 7-80 years). Biopsies were designated as "antral/antral nodules" (61%), and the rest were labeled "gastric/random stomach" (39%). Nine cases (3%) exhibited absence of gastrin-positive endocrine cells; one of those showed endocrine cell hyperplasia by chromogranin staining. CONCLUSIONS: Pathologists should be aware of the histologic features of early AMAG and meticulously analyze tissue regardless of specimen labeling. Gastrin immunostain is a supplemental diagnostic tool when encountering inflamed antral-appearing specimens.

Primary Dedifferentiated Amelanotic Anorectal Melanoma: Report of a Rare Case.

Anorectal melanoma (ARM) is an uncommon aggressive malignancy that comprises 0.5% to 1.6% of all melanoma manifestations. Dedifferentiated melanoma is very rare with loss of all melanocytic differentiation markers and is usually seen in metastatic melanoma of cutaneous origin. In this article, we report the first case of primary dedifferentiated amelanotic ARM in a 68-year-old male who presented with anal discomfort that was initially treated as inflamed hemorrhoids. Physical examination revealed a large protruding anal mass, biopsy of which showed biphasic malignant tumor cells with distinct immunoprofiles: a superficial spindled/sarcomatoid component positive for SOX10 and S100, and a deeper epithelioid/rhabdoid component positive for desmin, AE1/AE3, and EMA. Both components were negative for HMB-45, Melan-A/MART-1, c-Kit, and other lineage markers. Molecular analysis by polymerase chain reaction demonstrated wild-type BRAF and KRAS genes. A diagnosis of dedifferentiated ARM was made based on the coexistence of a differentiated component (spindled: S100 and SOX10 positive) and a dedifferentiated component (epithelioid: all melanoma markers including S100 and SOX10 negative). Shortly afterwards, the patient developed extensive pulmonary and liver metastases and expired 20 days after the diagnosis was rendered, reinforcing the highly aggressive nature of this disease entity.

ALK Rearrangements Are Infrequent in Cellular Blue Nevus and Deep Penetrating Nevus.

Recent studies have identified kinase fusions in Spitzoid melanocytic neoplasms, and approximately 10% of Spitzoid neoplasms harbor anaplastic lymphoma kinase (ALK) rearrangements and corresponding ALK immunoreactivity. Deep penetrating nevi (DPN), a subset of melanocytic neoplasms, have histologic and immunohistochemical overlap that have historically supported classification of DPN with blue/cellular blue nevi (CBN). However, HRAS mutations have rarely been detected in DPN, thereby also linking them to Spitz nevi. The purpose of this study was to see if DPN or CBN possess ALK rearrangements, thereby providing more evidence that these melanocytic lesions may be pathogenetically related to Spitzoid neoplasms. Using ALK immunohistochemistry as a surrogate for ALK rearrangement, the authors examined 26 DPN, 30 CBN, and 4 conventional blue nevi. ALK immunoreactive cases underwent fluorescent in situ hybridization to investigate for the presence of ALK gene rearrangement. Patchy and focal ALK immunostaining was found in only 1 case of DPN (1/26, 3.8%). Seven cases of CBN (7/30; 23%) showed ALK immunostaining (6 focal/patchy, 1 strong and diffuse). Fluorescent in situ hybridization using ALK break-apart probes showed various degrees of gain of 2p23 and rare ALK break-apart signals. Four CBN showed ALK rearrangement in 2%-4% of cells. Two cases of CBN showed gain of 2p23 in 10%-20% of cells. In our study, ALK rearrangements are uncommon in both CBN and DPN, making ALK an unlikely driver in tumorigenesis and classification of these melanocytic variants. However, our study did identify ALK molecular changes and immunohistochemical staining patterns that have not been previously described in CBN or DPN.

Naegleria fowleri That Induces Primary Amoebic Meningoencephalitis: Rapid Diagnosis and Rare Case of Survival in a 12-Year-Old Caucasian Girl.

Primary amoebic meningoencephalitis (PAM) is a rare and almost always fatal disease that is caused by Naegleria fowleri, a freshwater thermophilic amoeba. Our case involves an adolescent female who presented with fever of unknown origin. A lumbar puncture was performed, and the Wright-Giemsa and Gram stained cerebrospinal fluid (CSF) cytospin slides showed numerous organisms. Experienced medical technologists in the microbiology and hematology laboratories identified the organisms as morphologically consistent with Naegleria species. The laboratory made a rapid diagnosis and alerted emergency department care providers within 75 minutes. The patient was treated for PAM with amphotericin, rifampin, azithromycin, fluconazole and aggressive supportive therapy including dexamethasone. The Centers for Disease Control and Prevention (CDC) was contacted, and miltefosine, an investigational medication, was started. Additional treatment included an intraventricular shunt and controlled hypothermia in order to mitigate potential cerebral edema. Our patient is a rare success story, as she was diagnosed swiftly, successfully treated, and survived PAM.

111In-Pentetreotide Imaging in Diffuse Idiopathic Neuroendocrine Hyperplasia of the Lung.

Diffuse idiopathic endocrine neoplasia of the lung (DIPNECH) is a rare disease characterized by proliferation of neuroendocrine cells in the bronchial wall. Less than 20 cases of DIPNECH have been reported in imaging literature. We present here a case of histopathologically diagnosed DIPNECH with diffusely increased In-octreotide uptake in both lungs.

Femoral head and neck excision in cats: medium- to long-term functional outcome in 18 cats.

OBJECTIVE: To assess the medium- to long-term functional outcome of cats after femoral head and neck excision (FHNE) using an owner-completed questionnaire. METHODS: Cats that had FHNE and were free of other orthopaedic or medical conditions that could affect their mobility, other than the studied coxofemoral joint(s), were included. A specific owner-completed questionnaire was used at a minimum of 4 months postoperatively. The questionnaire assessed the ability of the cats to perform normal feline activities, change of demeanour or behaviour, the necessity for long-term analgesia and the time taken to resume normal activities. RESULTS: Eighteen cats had undergone uni- or bilateral FHNE and met the inclusion criteria. All but one cat could perform normal feline activities without or with slight difficulty at follow-up. The aforementioned cat had notable, persistent difficulty in climbing. The majority of the cats took between 1 and 2 months to resume normal activity. No change in demeanour or behaviour was noted in any of the cats and none of the cats required long-term analgesia. CONCLUSIONS AND RELEVANCE: Based on the owner-completed questionnaire, cats have good-to-excellent medium- to long-term functional outcome after adequately performed FHNE.

Trans-iliac pin/bolt/screw internal fixation for sacroiliac luxation or separation in cats: six cases.

Trans-iliac pin, bolt or screw stabilisation was performed on six cats with sacroiliac (SI) luxation and separation. For the purpose of this study, SI luxation is defined as the separation of the iliac wing from the sacrum without fracture of the sacral or iliac wing; SI separation is defined as the separation of the iliac wing from the sacrum secondary to fracture of the sacrum and/or the iliac wing. Complications, surgical time and medium-to-long-term outcome were assessed by a retrospective review of the clinical records and owner questionnaires. Postoperative reduction of the SI joint was good-to-excellent in all cases and the outcome was considered good-excellent in all cats apart from one, where the trans-iliac bolt migrated dorsally from the iliac wing. This cat had bilateral SI luxations. Based on our results, trans-iliac fixation of SI luxation/separation is associated with good clinical outcome and should be considered as a treatment option in unilateral SI luxation in cats. Caution should be exercised in the use of trans-iliac pin/bolt as the sole method of stabilisation in bilateral SI luxations.