Epithelioid Fibrous Histiocytoma With Dot-Like Perinuclear ALK Expression and PRKAR2A-ALK Fusion.

Epithelioid fibrous histiocytoma (EFH) is a rare, benign, cutaneous neoplasm. This fibrohistiocytic tumor was once believed to be a variant of fibrous histiocytoma, but EFH is now known to be a distinct entity based on the presence of ALK gene rearrangements in most cases. The pattern of immunohistochemical expression of ALK in EFH in the literature thus far describes both granular cytoplasmic staining and nuclear staining. We present a case of EFH with dot-like Golgi pattern perinuclear ALK expression, a previously undescribed staining pattern. We surmised this unique staining pattern could be due to a novel fusion partner, and using FISH, we confirmed a rearrangement of the ALK (2p23) locus. Further investigation with whole transcriptome sequencing led to the discovery of PRKAR2A-ALK fusion, and the function of this fusion partner reflects a Golgi-predominant localization of the protein. Attention to the distinct immunohistochemical pattern of ALK expression may provide clues to the function of the fusion partner.

Giant cell tumor of the bone presenting as swelling of the thumb.

We describe a giant cell tumor in a 74-year-old man who presented with pain and swelling in the left thumb. Radiographs of the left hand showed some lytic changes at the tip of the thumb. Magnetic resonance imaging suggested the diagnosis of giant cell tumor, which was later confirmed by biopsy. A good treatment response was achieved with disarticulation of the interphalangeal joint and amputation of the distal phalanx of the left thumb.

Extraocular Sebaceous Carcinoma Presenting As a Nodule over the Zygoma.

We describe a case of sebaceous carcinoma (SC) in a 75-year-old man who presented with a rapidly growing nodule on the left cheek for four weeks. A 75-year-old man presented with a crusted non-tender nodule on the left cheek that had been present for six months. The nodule showed rapid growth in the four weeks before the visit. Shave biopsy of the lesion was reported as SC. SC is a rare but aggressive type of skin cancer that can develop from any sebaceous gland in the body. However, it mostly occurs in the eyelids. In this case, SC developed on the left cheek of the patient, which is an atypical presentation. Extraocular variant of SC has a greater potential to metastasize and has a lower survival rate compared to ocular variant. Early and accurate diagnosis followed by a wide excision surgery or Mohs micrographic surgery carries a favorable prognosis. Early detection of extraocular variant of SC may be difficult. It is imperative for primary care providers to order a histopathology examination to investigate a rapidly growing mass in the head and neck region where there are numerous sebaceous glands.

Neoadjuvant Cabozantinib in Renal-Cell Carcinoma: A Brief Review.

Nephrectomy is the reference-standard treatment for renal-cell carcinoma (RCC). For patients with unresectable disease, tumor may be shrunk by using chemotherapy, thereby permitting surgical resection, which can be curative. We provided neoadjuvant cabozantinib, the preferred tyrosine kinase inhibitor for advanced RCC of poor and intermediate risk, to two patients with initially unresectable RCCs. In both patients, this led to tumor shrinkage of > 50% after 4 months of therapy, which permitted surgical resection. Both tumor specimens also showed strong pathologic tumor response. The robust responses observed with cabozantinib, even at reduced doses, suggest it to be an effective neoadjuvant option in RCC. Our novel experience with neoadjuvant cabozantinib, combined with our review of the use of cabozantinib in RCC, indicates that providing preoperative cabozantinib to facilitate potentially curative surgical resection has good results and should be further explored.