Juan Carlos Sánchez España & Roberto Secondi
We present the case of an ulcerative lesion of the eyelid as first presentation of type A lymphomatoid papulosis (LP) in a young adult. LP is a rare cutaneous lymphoproliferative disease with a risk of associated systemic or cutaneous lymphoma.
Mariel Angelou Parulan, Gangadhara Sundar, Yew Kwang Ong, Tseng Tsai Yeo, Victor Lee & Miriam Santiago Kimpo
We report a case of a 2-year-old female who presented with bilateral progressive proptosis, visual loss, nasal obstruction, and breathing difficulty. Magnetic resonance imaging revealed a large sino-orbital mass that was extending to the orbital apex and skull base. An initial diagnosis of rhabdomyosarcoma was made elsewhere on the basis of the presence of round and spindle cell tumor. Subsequent biopsy with immunohistochemical staining was positive for nuclear staining with β-catenin, shifting the diagnosis to a myofibroblastic tumor, favoring desmoid-type fibromatosis. With image guidance, near complete excision of tumor was performed by a multidisciplinary team, while respecting danger zones such as the skull base and the optic nerve. Following a recurrence over 2 months, additional excision was performed with a 6-month treatment of methotrexate and vinblastine. Desmoid tumor is a rare form of soft tissue tumor uncommonly seen in the orbital area. Although benign, it is known to be recurrent and infiltrative. Few data are known and further information will aid in the management of these tumors.
Conger, Jordan R.; Grob, Seanna R.; Tao, Jeremiah
An 87-year-old woman presented with worsening right facial and periocular pain due to a large facial mass. She was diagnosed with squamous cell carcinoma 1.5 years before and received radiation therapy at an outside institution. Examination revealed a large 10 × 11 cm ulcerative facial lesion over the right temporal region extending to the lateral canthus with proptosis (Fig. A). Silver nitrate can be seen atop the lesion. Extraocular motility showed severe limitation in all directions. Imaging demonstrated tumor in the orbit, through the skull base, and down to the temporal lobe of the brain. Histopathologic analysis from previous biopsy demonstrated a well-differentiated invasive squamous cell carcinoma with keratoacanthomatous features. Given the extent of the lesion, poor overall physical condition, and advanced age, the patient opted for nonoperative care. In an attempt to palliate this invasive tumor, the patient received 8 infusions of pembrolizumab (Keytruda; anti-PD1, immunotherapy agent). Six months after, external examination showed significant regression of the lesion with cicatrization down to the temporal fossa with an exposed, escharred lateral zygomatic arch and lateral frontal bone (Fig. B).
Wolkow, Natalie; Freitag, Suzanne K.
Balloon cell nevi are rare variants of nevocellular nevi in which greater than 50% of nevus cells are large and foamy. They are most common in patients under age 30, affecting males and females equally when found in skin. The balloon changes are attributed to defects in melanogenesis that lead to malformed premelanosomes and degenerative changes. Clinically they are indistinguishable from more common nevi. Care needs to be taken in assessing for nuclear atypia in melanocytic lesions with balloon cells as some melanomas may display balloon cell changes.
Ediriwickrema, Lilangi S.; Liu, Catherine Y.; Kikkawa, Don O.; Korn, Bobby S.
The authors describe a clinical case whereby a patient with metastatic cutaneous melanoma developed eyelash poliosis after undergoing treatment with combination immunotherapy with ipilimumab and nivolumab.
Everett, Lesley A.; Copperman, Thomas S.; Kersten, Robert C.
Ductal carcinoma of the lacrimal gland is a very rare and aggressive neoplasm, with clinical and histopathologic similarities to salivary ductal carcinoma. Of the 25 previously reported cases, 2 patients had metastases to local lymph nodes confirmed on pathologic examination. The authors now report the clinical presentation, histopathologic and immunohistochemical features, and the treatment of a third patient with lacrimal gland ductal carcinoma with spread to local lymph nodes. In contrast to ductal carcinoma, lymph node involvement has not been reported in the largest series of adenoid cystic carcinoma, a much more common lacrimal gland malignancy. This case highlights the need for possible lymph node surveillance in patients with lacrimal gland ductal carcinoma.
Bradfield, Yasmin; Burkat, Cat N.; Albert, Daniel M.; Potter, Heather A.D.
A 7-year-old healthy girl presented for an evaluation of a left vascular scleral mass. The lesion appeared spontaneously with no history of trauma, coagulopathy, or topical medication use. It was nontender, enlarging, and did not extend intraocularly. Her OS vision was 20/20, and the remainder of her eye examination was normal. Evaluation of the ocular mass included B-scan ultrasound, ultrasound biomicroscopy, anterior segment optical coherence tomography (OCT), and orbital MRI. The anterior segment OCT demonstrated vessels within the mass with no defined capsule. The orbital MRI confirmed a lesion isolated to the scleral layers of the globe, with low blood flow. The patient had a partial response to oral propranolol. Because the lesion vessels began to extend into her corneal endothelium, there was a concern for malignancy. A biopsy confirmed a benign intrascleral capillary hemangioma. Discontinuation of the propranolol demonstrated stability of the lesion 6 months later.
Wolkow, Natalie; Jakobiec, Frederick A.; Habib, Larissa A.; Freitag, Suzanne K.
Purpose: To highlight the histopathologic diagnostic challenges of small-cell predominant extranodal nasal-type natural killer/T-cell lymphoma (ENTNKT) of the orbit.
Methods: Retrospective chart review and histopathologic study with immunohistochemistry and in situ hybridization of 3 cases.
Results: Three cases of ENTNKT presented to the Mass Eye and Ear emergency room as orbital cellulitis over 1 year. The first case was unusual in that there was a predominance of small cells, giving the ENTNKT the histopathologic appearance of a nonmalignant inflammatory process. This challenging case is juxtaposed alongside 2 other cases, which exhibited the more typical lymphomatous microscopic appearance.
Discussion: ENTNKT can extend into the orbit from the adjacent sinuses or rarely arise primarily in the orbit. A diagnosis is typically made with a biopsy. Occasionally, however, the histopathologic diagnosis can be elusive when a predominance of small lymphomatous cells that are virtually indistinguishable from non-neoplastic inflammatory cells is present. Demonstration of CD56 positivity by immunostaining and in situ hybridization for Epstein–Barr virus are essential in confirming the diagnosis.
Conclusions: ENTNKT should be considered both in the clinical and histopathologic differential diagnoses of orbital infections and idiopathic inflammations (pseudotumor).
Jamshidian-Tehrani, Mansooreh; Nabavi, Amin; Taghavi, Mostafa; Sharif-Kashani, Shervin; Kasaee, Abolfazl; Sadeghi-Tari, Ali; Mirhosseini, Mohammad; Nowroozzadeh, M. Hossein
Purpose: To evaluate the clinical and ultrasonographic response of periocular infantile capillary hemangioma during treatment with oral propranolol.
Methods: Patients with infantile periocular hemangioma and visual or cosmetic concerns were enrolled in this prospective interventional case series. Propranolol was given at a dose of 2 mg/kg per day for at least 6 months. Evaluation of treatment response was performed at month 3 (time point 1) and month 6 (time point 2). Gray scale ultrasonography and color Doppler imaging were performed at baseline and month 3.
Results: Thirty-one patients with mean age of 4.1 ± 2.3 months were eligible for analysis. Complete or near complete clinical resolution was observed in 4 patients (12.9%) at time point 1 and 21 patients (67.7%) at time point 2. Longitudinal diameter, transverse diameter, thickness, arterial peak systolic velocity, and end diastolic velocity reduced significantly from baseline to 3-month follow up. Complete clinical response at time point 2 was significantly higher in patients with peak systolic velocity reduction >50% from baseline to month 3 than patients with peak systolic velocity reduction of 10% to 50% and <10%.
Conclusions: Propranolol is safe and effective for infantile periocular hemangioma. Ultrasonography and color Doppler imaging are useful modalities to monitor and predict the treatment response.
Tieger, Marisa G.; Jakobiec, Frederick A.; Ma, Lina; Wolkow, Natalie
Purpose: A case of a small benign storiform fibrous tumor of the conjunctival substantia propria is described to clarify the category of fibrous histiocytoma. In addition, a comparison of the various spindle cell tumors of the conjunctival substantia propria is explored.
Methods: The patient underwent a complete tumor excision, and the specimen was analyzed by histopathologic and immunohistochemical investigations.
Results: A cellular mass, composed solely of spindle cells in a storiform pattern without a component of histiocytic cells, was found beneath an undisturbed nonkeratinizing squamous epithelium and was separated from the epithelium by a grenz zone of uninvolved collagen. The lesion was sharply demarcated but not encapsulated. The Masson trichrome stain revealed scant deposition of intercellular collagen. The reticulin stain displayed numerous and delicate wiry fibers between the tumor cells and encircling capillaries. The Alcian blue stain demonstrated faint positivity in the interstitium. Immunohistochemistry revealed positivity for vimentin, factor XIIIa, smooth muscle actin, CD10, and CD45. Negative stains were obtained for CD34, CD56, S100, desmin, and Ki67.
Conclusions: The broad term of fibrous histiocytoma should be reserved for deep fibroblastic spindle cell tumors (e.g., those of the orbit) that display an aggressive behavior. More benign superficial spindle cell tumors of the dermis are now preferentially characterized as dermatofibromas. It is suggested that equally benign epibulbar tumors should no longer be designated as fibrous histiocytomas but rather as benign storiform fibrous tumors. Tumors completely composed of polygonal histiocytoid (epithelioid) cells that are CD34+ should be excluded from the benign storiform fibrous tumor category. Positive smooth muscle actin and factor XIIIa staining in conjunction with negative staining for CD34 and desmin in the current spindled tumor cells are findings consistent with those of cutaneous dermatofibromas. Both the epibulbar and dermal spindle cell lesions have displayed an indolent and nonaggressive behavior. Microscopically they contain a high proportion of dendrocytic stellate cells that are either factor XIIIa+ or XIIIa−. Given the anatomic differences between the dermis and conjunctiva, the term dermatofibroma is inappropriate for the current tumor; instead the term benign storiform fibrous tumor has been proposed for superficial tumors of the conjunctiva.