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.
Hind M. Alkatana, Azza M.Y. Maktabi
Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder that has been found in all continents and racial groups in relation to faulty repair of DNA with sun exposure. Several cutaneous and ocular tumors have been described in relation to XP including fibrous histiocytoma (FH). The diagnosis of conjunctival FH is challenging owing to the rarity of this tumor and the diversity of its classification into benign, locally aggressive and malignant. We are describing a recurrent FH exhibiting a locally aggressive behavior in a child with history of XP. Detailed histopathological features are presented with literature review.
Ramsey, Jonathan K.; Chen, James L.; Schoenfield, Lynn; Cho, Raymond I.
Undifferentiated pleomorphic sarcoma is a malignancy of mesenchymal origin, which was previously known as malignant fibrous histiocytoma. It is known to occur on rare occasion as a primary orbital tumor, but no known cases of metastatic orbital involvement have been reported since 2002, when the reclassification of these tumors took place. The authors report a patient who presented with a metastasis to the left orbit 2 years after undergoing treatment of a high-grade undifferentiated pleomorphic sarcoma of the right thigh. Histopathology of the orbital mass was similar to the primary tumor biopsy prior to neoadjuvant chemotherapy and radiation. The appearance was markedly altered in the subsequent excisional tissue, which showed treatment changes. Immunohistochemistry and genetic testing also supported the metastatic nature of the orbital lesion. The patient’s tumor progressed rapidly despite systemic targeted therapy and orbital exenteration was performed. At 2 years follow up, the patient remained without evidence of tumor recurrence in the socket.
Santamaria, Joseph, A., M.D.; Gallagher, Colin, F., M.D.; Mehta, Aditya, M.D.; Davies, Brett, W., M.D.
Fibrous histiocytoma is a soft tissue tumor with cells resembling both fibroblasts and histiocytes. Occasionally in the orbit, they rarely arise in the lacrimal sac. Similar to prior cases described, the patient presented with symptoms of epiphora and a slowly enlarging mass inferior to the right medial canthus. Imaging demonstrated a circumscribed 1.2 cm × 1.1 cm × 1.1 cm mass within the lacrimal sac without surrounding bony destruction. Grossly, the tumor appeared homogenous and rubbery. Pathologic study results from the case demonstrated zones of hypercellularity and hypocellularity with a fibrous appearance, admixed with spindle cells and collections of foamy histiocytes. A diagnosis of benign fibrous histiocytoma was rendered, with the patient experiencing a complete resolution of symptoms at subsequent follow up. To the authors’ knowledge, this is the first reported solitary case report of a fibrous histiocytoma of the lacrimal sac in a pediatric patient.
Soon, Alexander K.; Brownstein, Seymour; O’Connor, Michael; Iordanous, Yiannis
Fibrous histiocytoma is a mesenchymal tumor that can be classified as benign, locally aggressive, or malignant. A 15-year-old man was assessed for a superotemporal limbal conjunctival mass in the left eye increasing in size for 2 months. The lesion was initially flat, and gradually developed into a round, elevated, and fleshy-pink lesion (Fig. 1A) with freely mobile overlying conjunctiva and adherent to the underlying sclera with extension into the peripheral cornea. Examination with ultrasound biomicroscopy revealed a dome-shaped lesion with a maximum radial length of 8.17 mm and radial height of 2.75 mm (Fig. 1B). Histopathological examination disclosed cells with both histiocytic and fibrocytic morphology (Fig. 1C), which stained positive with vimentin (Fig. 1D), factor XIIIa (Fig. 1E), and CD163 (Fig. 1F), consistent with a diagnosis of fibrous histiocytoma extending into the adjacent sclera. Staining with Ki67, Langerin (CD207), S100, and CD1a were negative; a single mitosis was noted. The lesion was surgically excised and the underlying sclera was cauterized. Five months postoperatively, there was minimal conjunctival scarring at the surgical site with no evidence of recurrence.
Pavindran Gounder, Minh Lam, Carl Vinciullo & Jean-Louis de Sousa
A 68-year-old gentleman presented with a lesion that resembled a pyogenic granuloma in his inferior fornix. The lesion was excised and biopsy demonstrated a proliferation of malignant spindle cells. Three weeks following initial excision, the lesion recurred and was removed via wedge excision of the eyelid. Definitive clearance was achieved through Mohs micrographic surgery. The patient received adjuvant postoperative radiotherapy and remains disease-free. This case demonstrates the need to consider sinister pathology in the setting of recurrent periocular lesions.