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Sclerosing angiomatoid nodular transformation (SANT) of the spleen is an uncommon primary benign condition with specific histopathological characteristics. The majority of the patients are usually asymptomatic and present for treatment after an incidental finding on a routine abdominal imaging. The differential diagnosis includes other benign tumours, as well as primary malignancy or metastases of unknown origin. We report a 55-year-old woman who was presented to the surgical team of our hospital for splenectomy after the investigation of an incidental splenic lesion led to the diagnosis of SANT. The pathology report of the specimen repeated the initial diagnosis. The patient has not yet presented any complication or recurrence of the disease 8 months after the operation. From such infrequent cases, the most valuable conclusion which can be yielded is that the more the number of patients reported, the more the knowledge about the disease is broadened.

Treatment of dermatofibromas, beyond surgical excision, has remained inadequate and elusive. Nonsurgical treatment options are desired by both patients and physicians. Erbium glass lasers are known for targeting and remodeling the dermis but have not yet been studied for the treatment of dermatofibromas.To evaluate the efficacy and safety of the fractional 1540-nm erbium glass laser for treatment of dermatofibromas.Thirty-five patients representing 44 dermatofibromas completed 2 consecutive monthly treatments with a non-ablative, fractional 1540-nm erbium glass laser and were evaluated at 4, 8, and 12 weeks after the initial treatment. Dermatofibromas were evaluated using patient surveys given before and after the treatments.By week 4, patients reported improvement in color and texture of the dermatofibromas. These improvements were durable through week 12. No complications were reported.This study demonstrates that the fractional 1540-nm erbium glass laser may be used to improve the color and texture of dermatofibromas and achieve positive patient reported outcomes after only 2 treatments. J Drugs Dermatol. 2022;21(11):1201-1205. doi:10.36849/JDD.6287.

Epithelioid fibrous histiocytoma (EFH) is a distinctive cutaneous neoplasm with a relatively variable morphological appearance. Recently, it has been shown that this tumor is molecularly characterized by ALK gene fusions. We report three EFHs with unusual histological presentation represented by a prominent/predominant spindle cell proliferation arranged in a variably storiform/whirling architectural pattern with or without stromal sclerosis. One of the cases closely resembled cellular fibrous histiocytoma. All three cases were immunohistochemically ALK-positive and were analyzed for ALK gene rearrangements using a next-generation sequencing-based assay (FusionPlex Sarcoma Kit, ArcherDx). Three novel fusions, namely AP3D1::ALK, COL1A::ALK, and LRRFIP2::ALK, were detected and further confirmed by FISH in all 3 cases and RT-PCR in 1 case. All patients were elderly (62-63 years) and presented with a solitary polypoid lesion on the extremities. The awareness of these morphological variants is important since it entertains a wide and slightly different differential diagnosis than conventional EFH. We also presented evidence that a clear separation of EFH from BFH in all cases may not be as straightforward as previously thought. The consistent ALK immunoexpression and the continually expanding scale of ALK gene rearrangements provide a useful tool to distinguish EFH from its histologic mimics.

Multinucleate cell angiohistiocytoma (MCAH) is a benign vascular and fibrohistiocytic (probably reactive) proliferation with peculiar multinucleate cells which most authors interpret as degenerated macrophages. Several clinical variants of MCAH have been described, some of them with brownish-appearing lesions clinically. However, no histologically identified pigment has been described in the cytoplasm of the multinucleate cells so far. We present a pigmented MCAH with cytoplasmic brownish pigment, which was positive with a Masson-Fontana stain and negative with an iron stain, consistent with melanin, in a 33-year-old woman with multiple papules and plaques on the right elbow, right jawline, and left flank.

Immunohistochemical staining can be of great utility in differentiating various cutaneous spindle cell neoplasms, particularly when the histomorphologic appearance of the lesions is inconclusive. Nuclear staining for ETS-related gene (ERG), a highly sensitive endothelial cell marker, has seldom been studied in the context of cutaneous spindle cell neoplasms. Little is known about its specificity for vascular differentiation. In this pilot study, immunohistochemical analysis for ERG was performed on 15 dermatofibromas (DF), 10 keloids, and 9 dermatofibrosarcoma protuberans (DFSP) tumors. Consistent nuclear expression of ERG was found in DF [100% (15/15) of the lesions demonstrated50% labeling of tumor cells with moderate to strong intensity]. However, ERG expression was largely absent in DFSP [89% (8/9) of the lesions demonstrating50% labeling staining, generally of mild intensity] and hypertrophic scars-keloids [80% (8/10) without expression]. On the basis of the results of this pilot study, immunohistochemical staining for ERG may prove useful in helping to differentiate DF from DFSP and hypertrophic scars in the context of partial biopsy sampling. If replicated in a larger number of samples, this finding could mitigate the use of costly sequencing panels and potentially avoid unnecessary reexcisions in certain contexts.

Epithelioid fibrous histiocytoma (EFH) is a cutaneous neoplasm driven by translocations of the anaplastic lymphoma kinase (ALK) gene, which can be demonstrated by immunohistochemical (IHC) analysis. We analyzed the performance of two ALK clones, D5F3 and ALK1, in a cohort of EFHs and described the range of architectural variation of these lesions. TFE3 IHC was performed in ALK-negative EFHs. We identified 21 cases of EFH, 76.2% of which showed an exophytic appearance and 19% displayed flat architecture. A well-developed epidermal collarette was present in 48% of all cases with just more than a third of all the exophytic lesions presenting as dermal-based nodules. ALK D5F3 expression was identified in 76.2% (16/21) of all cases, but only 68.8% were concordantly positive with the ALK1 clone, indicative of a false-negative stain with ALK1 in 31.2% of the cases. For the subset of cases showing positivity for the ALK1 clone, a marked decrease in the percentage of immunolabelled cells was identified when compared with D5F3 (5-50% vs. 100%, respectively). Five cases (23.8%) did not demonstrate ALK expression for either clone, with 3 of those cases showing nuclear positivity for TFE3 IHC and the remaining 2 cases being double negative (ALK-/TFE3-). In summary, we identified that the prototypically described exophytic appearance with epidermal collarette is present in only less than half of the cases. We also demonstrated that the ALK1 antibody is suboptimal in EFH and should not be utilized in this setting. A subset of ALK-negative cases express TFE3, but double-negative cases occur.

Objective The aim of the present study was to carry out a systematic review of available data regarding case reports and case series of oral and maxillofacial benign fibrous histiocytoma (BFH). Study Design A search strategy was performed using the PubMed, Web of Science, Scopus, and EMBASE electronic databases. Results Male individuals (56.89%) were more affected, with a mean age of 34.55 ± 20 years. The buccal mucosa (20.33%) represented the most common site, with the clinical presentation of a painless (86.95%) nodule (98.03%). The clinical hypothesis of a fibroma was reported in most cases (31.57%). Radiographic presentation of intraosseous lesions showed multilocular radiolucent images (55.55%). Regarding the histopathologic features, the biphasic population of fibroblastic and histiocytic cells was seen in 21 cases (39.62%), and a spindle-shaped fibroblastic cell population organized into a storiform pattern was observed in 25 cases (47.16%). CD68 (n = 26) and vimentin (n = 25) showed immunoreactivity in all BFH cases in which they were used. All cases were treated with a surgical resection, and 8.10% recurred. Conclusions The current systematic review demonstrated that BFH represents a rare lesion that mainly affects the buccal mucosa of male individuals, and the treatment is mainly surgical with a good prognosis.

The diagnosis of cutaneous mesenchymal neoplasms remains challenging, due to a combination of overlapping histological features, the rarity of some diagnoses and often inadequate sampling in superficial biopsies. Here, we describe recent advances in cutaneous mesenchymal neoplasms. We discuss improvements in our understanding of the molecular pathogenesis of non-neural granular cell tumour, epithelioid fibrous histiocytoma, composite and retiform haemangioendothelioma and dermatofibrosarcoma protuberans. We also discuss recently described tumour types, including some discovered via molecular testing: EWSR1::SMAD3-rearranged fibroblastic tumour, clear cell neoplasm with MITF::CREM rearrangement and melanocytoma with CRCT1::TRIM11 rearrangement, and some discovered via traditional histopathology: superficial CD34-positive fibroblastic tumour, plexiform myofibroblastoma and clear cell neoplasm with melanocytic differentiation and ACTIN::MITF translocation.

Dear Editor, Morphea profunda (MP) is a chronic autoimmune disease, a subtype of localized scleroderma that presents clinically as local discomfort due to the impairment of skin motility (1). Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue neoplasm that not only infiltrates the dermis and subcutaneous tissue, but can also affect the muscles and bones with finger-like extensions, usually present on the trunk and the proximal extremities (2). DFSP is known for its indolent clinical course, locally aggressive behavior, and high local recurrence rates, but relatively low risk of metastatic spread (2). DFSP frequently arises in middle-aged adults, affecting both sexes equally with an incidence of 4 per 1,000,000 people (3). We report the case of a 39-year-old female patient who first presented to our clinic at the age of 20 years due to a brownish atrophic coin-sized lesion appearing on the left side of the abdomen. Medical reports indicated that biopsies had been performed previously on 3 occasions, and histopathologic findings confirmed the diagnosis of MP. The aforementioned lesion on the abdomen had been growing slowly over the years, and the patient finally visited our clinic 15 years later after noticing two palpable nodules developing within the affected skin (Figure 1, A, B). Clinical examination revealed an indurated ill-defined plaque measuring 10 cm with partially atrophic surface and 2 centrally located palpable nodules measuring between 3 and 5 mm. A deep biopsy of the lesion was performed, and histopathology and immunohistochemical analysis of CD34 expression confirmed the diagnosis of dermatofibrosarcoma protuberans (Figure 1, C, D). Computed tomography scans of the thorax, abdomen, and pelvic region were subsequently performed, revealing no further disease progression. Complete excision of the tumor was performed and followed by wide scar re-excision due to narrow surgical margins of only 1 mm. No further disease progression or recurrences have been noted during the follow-up, and the patient has been disease-free for one year postoperatively. Although the etiology of DFSP is unknown, trauma has been hypothesized as a predisposing factor. It usually presents on the trunk and the proximal extremities (4). Patients usually report disease progression over a long period of time, ranging from several months to years. The tumor is associated with variable color changes, even proximal skin discoloration, and often presents with a slowly growing indurated dermal plaque or firm nodule attached to the skin (4). Clinically, it can be difficult to distinguish DFSP from a wide number of diagnoses, including morphea, idiopathic atrophoderma, atrophic scar, anetoderma, lipoatrophy, cellular dermatofibroma, fibrosarcoma, malignant fibrous histiocytoma, atypical fibroxanthoma, desmoplastic melanoma, Kaposi sarcoma, and solitary fibrous tumors (5). Immunohistochemistry staining for CD34 cells can be helpful in differentiation, since spindle cells stain positively in DFSP (6). Due to alteration of dermal collagen, histopathological differential diagnoses of DFSP includes lichen sclerosus, atrophic scars and keloids, as well as morphea (7), atrophic dermatofibroma, and undifferentiated pleomorphic sarcoma (6). The mainstay of DFSP treatment is tumor excision performed either by wide local excision or Mohs surgery and having surgical margins between 1 and 5 cm. Several studies have confirmed that patients treated with the Mohs technique have significantly lower recurrence rates (8). Due to the high number of unsatisfactory primary excisions, wide free surgical margins are important for disease control (3). Radiotherapy might be considered as a therapeutic option for inoperable tumors or relapses, as well as an adjuvant therapy after primary excision or re-excision with positive margins (8). Furthermore, recent findings indicate positive therapeutic efficacy after administration of imatinib mesilat - a tyrosine kinase inhibitor due to over expression of PDGFβ (9). Clinical follow-up of patients with DFSP after tumor excision should be performed every six months for the first five years, followed by yearly intervals thereafter for up to 10 years (3). Previous case reports have claimed that the diagnosis of DSFP is commonly delayed as a result of slow tumor growth and nonspecific initial clinical findings (10). To the best of our knowledge, our case is the first description in the literature of DFSP developed within a MP plaque. We speculate that trauma from repeated punch biopsies taken from the sclerotic morpheaform plaque may represent the trigger for the development of the DFSP. Another notable clinical challenge was the surgical excision itself, since the majority of cases presented in literature mentioned unsatisfactory resection margins and a high risk of local disease recurrence. Although complete excision of the neoplasm was performed, re-excision was performed in order to provide wider resection margins. Surgical resection remains the main treatment for dermatofibrosarcoma protuberans, with the main challenge being the achievement of clean excision margins. Proper management of the disease and continuous follow-up are important in order to prevent local recurrence of dermatofibrosarcoma protuberans or its potential metastases.

Dermatofibromas (DFs) are benign lesions that typically present as firm papules or nodules on the legs of young- to middle-aged adults. DFs are histologically characterized by a dermal proliferation of spindled fibrohistiocytic cells forming intersecting fascicles and showing collagen entrapment. The palisading variant of DF was first described in 1986 and often presents as a dome-shaped nodule on the digits. Histologically, palisading DFs demonstrate central areas of nuclear palisading in parallel rows resembling Verocay bodies, with more typical areas of DF located peripherally. We report a case of a 33-year-old Hispanic woman who presented with a history of an asymptomatic, slow-growing lesion on her left arm present since her teenage years. Physical examination revealed a solitary, firm brown-to-white 5-mm papule on the left upper arm. A biopsy was performed and revealed a proliferation of spindle cells palisading around areas of hyalinized collagen. The many histologic variants of DF can sometimes create diagnostic confusion. Previously described cases of palisading DFs in the literature showed palisading resembling Verocay bodies and thus raising concern for a schwannoma or other neoplasm that display a "rippled" pattern. Our case is unique in that the architecture of the palisading areas instead resembled a necrobiotic granulomatous process such as granuloma annulare or a rheumatoid nodule at low power.

Purpose The purpose of this study was to describe the computed tomography (CT) and magnetic resonance imaging (MRI) features of sclerosing angiomatoid nodular transformation (SANT) of the spleen and correlate imaging features with those obtained at histopathologic analysis. Materials and methods A total of 18 patients (9 men, 9 women; mean age, 42.2 ± 10.7 [standard deviation (SD)] years; range, 23–59 years) with histopathologically confirmed SANT were retrospectively evaluated. The presenting symptoms, gross pathologic changes, and histopathologic and correlative immunohistochemical results were recorded. CT (n = 8) and MRI (n = 12) features were analyzed by two radiologists and included number, size, shape, boundary, attenuation, signal intensity, and enhancement patterns. Results Seventeen patients (17/18; 94%) had a single SANT without specific clinical symptoms and one patient (1/18; 6%) had multiple SANTs with left-upper-quadrant bloating and pain. The largest lesion diameter exceeded 3 cm. On plain CT images, SANTs were slightly hypoattenuating in seven patients (7/8; 88%), isoattenuating in one patient (1/8; 13%), and contained calcification in two patients (2/8; 25%). On T2-weighted MR images, SANTs displayed hypointensity in ten patients (10/12; 83.3%), isointensity in one patient (1/12; 8%) and hyperintensity in one patient (1/12; 8%). On T2-weighted images, stellate or scattered fibrous scars were observed in all patients (12/12; 100%). On diffusion-weighted images, SANTs appeared as heterogenous or homogeneous hypointense in 12 patients (12/12; 100%). Compared to out-of-phase images, SANTs displayed decreased local signal intensity on in-phase images in 12 patients (12/12; 100%). On enhanced CT and MRI images, SANTs had clear boundaries (17/18; 94%), oval (7/18; 39%) or lobular (7/18; 39%) shape, displayed progressive centripetal enhancement (18/18; 100%), spoke-wheel pattern (14/18; 78%), nodular enhancement (11/18; 61%), or delayed enhancement of central fibrous scar (9/18; 50%). Conclusions SANT of the spleen predominantly manifests as a solid, single, oval or lobular, and well-defined lesion with a fibrous scar and occasional calcification. Typical enhancement characteristics include progressive and centripetal enhancement, spoke-wheel pattern, nodular enhancement, and delayed enhancement of central fibrous scar. Hypointensity on T2- and diffusion-weighted images are due to hemosiderin deposition and fibrous tissue.