Solitary skin-colored nodule on a child’s face

Sir, A healthy, six-year-old boy presented with a slowly grown dome-shaped nodule on the mandibular angle region present for two years. The patient’s past medical and family history were unremarkable. A physical examination revealed a solitary, 1.3 × 1 cm, firm, painless, flesh-colored tumor (Fig. 1). Dermoscopy showed branching, serpentine vessels on a pink background (Fig. 2a). These features disappeared when slight pressure was exerted on the dermoscope and the tumor exhibited a central, white, structureless area (Fig. 2b). An excisional biopsy was performed. A microscopic examination showed a well-circumscribed, paucicellular dermal tumor composed of eosinophilic collagen bundles separated by clefts and forming a storiform pattern. Scattered fibroblasts were found among the collagen bundles. The overlying epidermis was slightly flattened (Fig. 3). The diagnosis of solitary sclerotic fibroma was established. Sclerotic fibroma (SF), also known as storiform collagenoma, is a rare benign skin tumor. It usually manifests itself as an asymptomatic, slowly growing, white-to-skin-colored papule or nodule [1]. It was first described in patients with Cowden’s disease, yet may also occur sporadically [2]. There were no mucocutaneous features of Cowden’s disease (tricholemmomas, oral fibromas, acral keratoses, palmar pits, and gingival and palatal papules) in the patient and her family members. Dermatofibroma, the main differential diagnosis of SF, usually exhibits hyperplastic changes of the epidermis instead of atrophy, and the boundaries of the lesion are unclear [2]. Only two papers have been published describing the dermoscopic findings of SF, consisting of a white background with peripheral arborizing vessels [3]. A white background may be related to an increased dermal collagen density. It is also described in dermatofibroma, typically with a peripheral pigmentation network. Although dermoscopy may improve the clinical diagnosis of SF, histopathological analysis is required.

Fibrous Histiocytoma of Anterior Ethmoidal Sinus: Case Report and Review of the Literature

Benign fibrous histiocytoma of the para nasal sinus is a rare tumor of this region which originates from mesenchymal cells. A 9-year-old girl presented with gradually enlarging mass above the medial canthus over 3 months. Orbital computerized tomography scan showed a round well-differentiated homogeneous mass in the anterior ethmoidal sinus. Histology and immunohistochemistry analysis after excision revealed proliferation of spindle-shaped fibroblasts in storiform pattern and histiocytes without mitosis and anaplasia. After 9 months from surgical excision, recurrence occurred which resulted in total excision again. This is the first reported case of benign fibrous histiocytoma involving the ethmoid sinus in Iranian people. Rare sinus tumor should be considered in the differential diagnosis of sinus tumors.

Case Report: Predominant Tubulointerstitial Lupus Nephritis or the Combination With IgG4-Related Disease?

Isolated or dominant tubulointerstitial lupus nephritis is rare. Here, we reported a 67-year-old man diagnosed with systemic lupus erythematosus (SLE) based on clinical and laboratory criteria, who was showing impaired renal function and non-nephrotic range proteinuria in the past 2 years. Renal biopsy showed almost normal glomeruli, but the tubulointerstitium showed “storiform” pattern with interstitial infiltration of IgG3 predominant plasma cells. Immunofluorescence showed linear and granular staining of IgG and C1q along TBM and interstitium. He started on medium dose of oral steroids and mycophenolate mofetil, which were gradually tapered. As a result, his renal function improved over a few days. Now, he continued on low dose steroids and mycophenolate mofetil with no evidence of relapse.

Atrophic Pigmented Dermatofibrosarcoma Protuberans: A Case Report and Literature Review

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma characterized by the proliferation of spindle cells arranged in a storiform pattern. Here we report a case of DFSP presenting as a bluish-black atrophic plaque. The tumor had the histopathologic characteristics of both the pigmented and atrophic variants of DFSP. We describe the histopathologic, molecular, and imaging features of this patient and review other cases of DFSP that have been reported in the literature. Early identification of this DFSP variant can help clinicians implement clinical management strategies to prevent recurrence.

Double-Stage Complete Removal of Dumbbell-Shaped Trigeminal Schwannoma: 3-Dimensional Operative Video

Intracranial trigeminal schwannomas are rare tumors.1-4 The aim of this 3-dimensional operative video is to present a double stage complete removal of a dumbbell-shaped trigeminal schwannoma. This is a 25-yr-old male with headaches, diplopia, and facial pain. The MRI shows a big tumor located at the level of the cerebellopontine angle, petroclival region, and middle fossa. Because of the size of the tumor and its growth within the cerebellopontine angle, we decided to operate the patient in two stages. For the first surgery, the patient was in a semi-sitting position, and a retrosigmoid approach was performed. The second surgery was performed 2 mo after the first operation in a supine position for a pretemporal transzygomatic approach. The pathological study was reported as a schwannoma, and the histological findings were spindle cell lesion with a storiform pattern and histiocytes. The patient evolved without neurological deficit after the surgeries, and the postoperative MRI shows a complete resection of the tumor. The patient gave the consent to use the images and surgical video. Preoperative imaging plays an important role in diagnosis and surgical planning.3-6 For these cases of trigeminal schwannomas with a large extension in the posterior fossa and middle fossa, we believe that the most prudent thing is to perform the surgery in 2 stages.3

Dermatofibroma Over The Scar – A Rare Entity

Dermatofibroma is a common benign dermal tumour of unknown aetiology. With varied clinical presentation mimicking keloid, desmoid tumour and leiomyoma, the diagnosis of Dermatofibroma sometimes become problematic from the clinician side. Here, we report a case of Dermatofibroma in a not so common site which was clinically diagnosed to be a keloid. But later, the lesion turned out to be a dermatofibroma on histopathological examination. In our patient, the lesion was a single smooth circumscribed nodule over the left side of the abdomen. The lesion had a linear scar on either side and on palpation; it was firm in consistency. It was initially diagnosed to be a keloid which even after multiple intra-lesional steroid injections, failed to show any results. This prompted us to search for an alternate diagnosis; hence lesion was excised and analyzed. The Histopathological examination revealed a circumscribed lesion in the dermis, composed of benign spindle-shaped cells arranged in a storiform pattern. These findings, as mentioned above, were consistent with a diagnosis of Dermatofibroma, which is a slow-growing tumour commonly seen in the extremities. The keloid like a presentation of Dermatofibroma, is one another example of how a similar morphological presentation may have two distinct diagnoses resulting in a delay in providing appropriate treatment.

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade fibroblastic mesenchymal tumor derived from the dermis. The aim of this retrospective analysis was to summarize the clinicopathological data from our cases and published cases to offer more evidence for the recognition of dermatofibrosarcoma protuberans (DFSP). A total of 6 breast DFSP patients who had received treatment in our hospital were retrospectively enrolled, and detailed clinicopathological data were gathered for analysis. The median age was 29.5 years (ranging from 17 to 42 years). Most cases presented a red or brown-red, mobile, well-circumscribed, protruding, breast mass (ranging from 1 to 3 cm). For histopathology, all cases (6/6) showed a storiform pattern of spindle cells that were positive for CD34 (6/6) and Vimentin (5/6) and negative for smooth muscle actin (0/6) and S-100 protein (0/6). The majority of patients (5/6) underwent wide local excision, with 2 cases treated with radiotherapy. With a median follow-up of 36 months, all 6 patients survived without recurrence or metastasis. The PubMed database was used to search for similar cases. Eventually, 36 cases were included in this review, while cases without detailed clinical information or not reported in English were excluded from the analysis. To summarize, DFSP of the breast is an extremely rare malignancy characterized by spindle tumor cells arranged in a storiform pattern and positivity for CD34. The core needle biopsy is one of the crucial methods for its preoperative diagnosis. Management of DFSP is mainly based on surgical excision. It is prone to local recurrence, so long-term follow-up is required.

Macroglossia in Beckwith-Wiedemann Syndrome Is Attributed to Skeletal Muscle Hyperplasia

Macroglossia is a common feature in patients with Beckwith-Wiedemann syndrome (BWS). The underlying cause of macroglossia in BWS remains unknown, and further histological studies are required to uncover its etiology. We present the case of a 5-year-old girl who was diagnosed with BWS and underwent partial tongue resection for difficulties in articulation and aesthetics. The keyhole-shaped partial resection revealed a harder posterior side than the anterior. Microscopically, the posterior side consisted of dense subepithelial eosinophilic areas composed of an abundance of tightly packed skeletal muscle fibers that were arranged in a fascicular or storiform pattern. BWS-associated macroglossia results from skeletal muscle hyperplasia, consistent with true macroglossia. Therefore, tongue resection may be beneficial for such patients. Further studies are required to develop personalized surgical interventions for each patient with BWS.

IgG4-related skin diseases: A brief review

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome which shares similar pathologic, serologic, and clinical features in the affected organs. Subacute development of a mass or diffuse enlargement of the organ is a common presenting feature. It is more commonly seen in middle-aged or older men. Lymphadenopathy is common in them. Tissue infiltration with lymphoplasmacytic collection, predominantly of IgG4-positive plasma cells, accompanied by “storiform” pattern of fibrosis, obliterative phlebitis, and increased tissue eosinophils are the hallmark histologic findings. Rapid response to systemic steroids is characteristic. If present, the typical cutaneous findings such as papulonodules or plaques in the head-and-neck region may serve as an initial clue to the underlying systemic involvement in IgG4-RD. Hence, dermatologists need to be aware of this entity for early recognition of underlying organ involvement and thus the prompt management.