Multinucleate Cell Angiohistiocytoma Research Articles

Pigmented Multinucleate Cell Angiohistiocytoma: Not So Infrequent?

To the Editor: In 2022, we described the first case of pigmented multinucleate cell angiohistiocytoma (MCAH), demonstrating that the pigment showed positivity with the Masson–-Fontana histochemical technique, while being negative with iron technique.1 At this time, no additional cases of this pigmented variant have been published. However, we have recently identified a second case of pigmented MCAH with similar staining characteristics by histochemical studies. The patient is a 58-year-old woman with a 3-year history of lesions on the back of her hands. The lesions were asymptomatic, although they had gradually increased in number and size. At the time of consultation, the patient had a total of 10 lesions, and a biopsy was performed on the largest lesion (5 mm in diameter). The histopathological study demonstrated a typical case of MCAH (Fig. 1A) with dermal fibrosis, numerous vessels, and polygonal multinucleated histiocytes (Fig. 1B) expressing CD68 (Fig. 1C), CD10, and FXIIIa. Some of the histiocytes showed cytoplasmic brownish pigment (Fig. 2A). This pigment did not stain with the Perls iron histochemical technique (Fig. 2B), but showed strong staining with Fontana-Masson (Fig. 2C), consistent with melanin.FIGURE 1.: A, Multinucleated cell angiohistiocytoma. At this magnification, the lesion appears as a conventional MCAH, with dermal fibrosis, prominent vasculature, and perivascular histiocytic infiltrate (Hematoxylin–-eosin ×40x). B, Some of the histiocytes exhibit angular polygonal conformation (Hematoxylin–-eosin ×20x). C, Expression of CD68 by the histiocytic component of the lesion (×100x).FIGURE 2.: A, Some of the histiocytic cells in the tumor contain brownish cytoplasmic pigment (Hematoxylin–-eosin ×400x). B, The pigment did not stain with the iron histochemical technique (×100x). C, The pigment was positive with the Fontana–-Masson technique (×200x).This finding of a second case in just over a year since the publication of the first one makes us think that, given the low frequency of this entity, the pigmented variant of MCAH may not be so exceptional. It is possible that the amount of pigment varies from case to case and that, therefore, many cases with mild amounts of pigment likely go unnoticed or are diagnosed without being published. Although in the discussion of our first case, we stated that the pigment was consistent with melanin, lipofuscin also stains with the Masson–-Fontana technique. Lipofuscin is a brownish lipid pigment that is considered a “"wear and tear”" or “"cellular aging”" pigment, because it is thought to result from the peroxidation of polyunsaturated lipids in subcellular membranes by free radicals. It is generally observed in cells with intense oxidative enzyme activity.2 In ultrastructural studies, lipofuscin deposits appear as cytoplasmic granules of varying sizes.3 Regarding this, we have performed additional techniques in both cases, including periodic acid-Schiff (PAS), Ziehl–-Neelsen, and Sudan black, which also stain lipofuscin.4 We have also examined the biopsy with an immunofluorescence microscope, becauseas it is known that lipofuscin shows autofluorescence. The pigment in both cases was negative by PAS staining, did not stain with Ziehl–-Neelsen, and did not show autofluorescence. Thus, it does not exhibit the staining or microscopic properties of lipofuscin. On the contraryHowever, both cases present properties consistent with melanin. We have not found any more cases published in the literature. There is a series of sclerosing hemangiomas from 1943, but the drawings of the microscopic observations suggest that they are lesions very different from MCAH.5 Additionally, the pigment demonstrated in some of the cases in that series was clearly identified as iron by its histochemical properties.5

Treatment Options for Multinucleate Cell Angiohistiocytoma: A Case Series and Review of the Literature.

Multinucleate cell angiohistiocytoma (MCAH) is an unusual fibrohistiocytic proliferation in the dermis with vascular hyperplasia. Numerous clinicopathological studies of MCAH have been published, but little has been written in relation to treatment. The aim of the present study is to review the therapeutical approaches for MCAH in the literature and to report the results of the different therapies followed at our institution. A literature review was conducted including all MCAH published cases that followed any therapy. Search terms included "multinucleate cell angiohistiocytoma" or "MCAH" and "treatment" or "therapy". Also, all cases of MCAH treated at our institution from 2010 to 2020 are reported. The literature search revealed 16 cases of MCAH treated with any of the therapeutic options. At our institution, 9 patients have been treated of MCAH between 2010 and 2020. Over 75% of them were female, and the median age was 56 years (range 47-73). More than 50% had lesions on the dorsum of the hands. Surgical excision was indicated in 2 patients, ablative CO2 laser was used in 1 patient and the 6 remaining ones followed vascular-targeted therapies (PDL and IPL). A satisfactory cosmetic result was achieved in all of them. We propose PDL therapy as a first-line treatment for MCAH since it achieves satisfactory esthetic results, while being well-tolerated. Selecting those amenable patients according to morphologic characteristics may be useful to avoid unsuccessful therapies. In MCAH with predominant fibrous stroma, surgery or CO2 laser may be the best option.

Multinucleate Cell Angiohistiocytoma: A Case Report and Unique Treatment Consideration

Introduction: Multinucleate cell angiohistiocytoma (MCAH) is a benign, yet exceedingly rare vascular and firbiohistiocytic proliferation. The lesions are typically asymptomatic and are commonly cosmetically displeasing to patients. 1 Previous reports have demonstrated success in treating MCAH with fractional ablative CO2 laser, argon laser, pulsed dye laser, and KTP lasers. 3 The scarcity of reported cases may suggest that the lesion is commonly misdiagnosed, and we report this case to highlight the characteristic features of MCAH with successful treatment with Neodymium YAG laser.
 Case Presentation: A 64-year- old female presented to our clinic with a 1- month history of multiple erythematous to violaceous lesions on her bilateral hands. The lesions were asymptomatic, but cosmetically displeasing for the patient. Past medical history and family history was noncontributory. Examination revealed several 2-6 mm well- circumscribed, erythematous, indurated smooth papules on the bilateral dorsal and in the second web space of the left hand. Shave biopsy of the lesions were consistent with MCAH. She was initially treated with topical steroids twice daily for 2 weeks, with no improvement. The patient received 3 treatments with Neodymium YAG, 4 weeks apart. This treatment was discontinued after 3 sessions due to the dramatic visible improvements in regards to induration and discoloration of the lesions.
 Discussion: MCAH is an extremely uncommon lesion, diagnosis is made on the basis of clinical examination, histopathologic and immunohistochemical analysis of the lesions. This case illustrates diagnosis and successful treatment of MCAH with Neodymium YAG and offers a unique treatment consideration for patients.

Giant plaque-type multinucleate cell angiohistiocytoma after total hip arthroplasty

Giant plaque-type multinucleate cell angiohistiocytoma after total hip arthroplasty

Successful treatment of multinucleate cell angiohistiocytoma in an adult male patient with intralesional corticosteroid and KTP laser

Successful treatment of multinucleate cell angiohistiocytoma in an adult male patient with intralesional corticosteroid and KTP laser

A case of multinucleate cell angiohistiocytoma in a 14-year-old boy showing two different clinical and histopathological findings

A case of multinucleate cell angiohistiocytoma in a 14-year-old boy showing two different clinical and histopathological findings

Multinucleate cell angiohistiocytoma: A clinicopathologic study of 62 cases and proposed diagnostic criteria.

Multinucleate cell angiohistiocytoma (MCAH) is an uncommon and likely underdiagnosed entity that is thought to be of vascular and fibrohistiocytic origin. We retrospectively reviewed all cases diagnosed as MCAH at the Yale Medicine Dermatopathology laboratory between 1 January 1990 and 1 September 2018. Sixty-two cases were retained. We performed immunohistochemistry on the ten most inflamed lesions found and assessed for a possible alteration within the Wnt/ß-catenin signaling pathway, involved in follicular induction in dermatofibroma. We subsequently established histologic diagnostic criteria to differentiate MCAH from its mimickers. MCAH affected both genders equally. The hands or fingers were affected in 51.6% of cases. We found the most specific histologic criteria to be: (a) presence of odd multinucleated fibroblasts, (b) presence of superficial parallel fibrosis, (c) presence and thickening of superficial papillary dermal vessels, and (d) absence of perifollicular fibrosis. As for immunoreactivity, we found positivity to CD138, CD163, and CD117 in the mononuclear inflammatory infiltrate. There was no histopathologic evidence of follicular induction, as can be seen in dermatofibromas, and no expression of nuclear beta-catenin as seen in dermatofibromas with follicular induction. This large case series establishes MCAH as a distinct clinical and histopathologic entity.

A case of multinucleate cell angiohistiocytoma in a 14‐year‐old boy showing two different clinical and histopathological findings

Multinucleate cell angiohistiocytoma (MCAH) is a rare cutaneous disease entity characterized by multiple red-to-brown or violaceous papules usually located on the acral regions, such as the face and the distal arms and legs. It affects elderly women more than men and rarely occurs at a young age. The exact pathogenic mechanism of MCAH is not yet clearly understood. We report an exceptionally rare case of a 14-year-old boy who presented with multiple asymptomatic erythematous papules and a single flat brownish plaque on the left chest. The brownish plaque lesion histologically showed proliferation of dilated small vessels in the upper-mid dermis and numerous oddly shaped multinucleate cells intermingled with lymphocytes and macrophages. The erythematous papules also showed dilated small vessels in the upper-mid dermis and multiple interstitial histiocytic infiltrations, but no multinucleate cells were detected. In immunohistochemistry studies, CD68 and vimentin staining were positive for both specimens. Based on the clinicopathological findings and immunohistochemistry studies, MCAH was diagnosed. To the best of our knowledge, this is the first case report of MCAH occurring in young age and showing two different clinical and histological phases at the same time.

Multinucleate cell angiohistiocytoma: Case report and literature review

Multinucleate cell angiohistiocytoma is a rare, vascular, fibrohistiocytic proliferation that has a benign but progressive course. The clinical presentation is that of grouped red-purple papules and nodules characteristically located on the lower extremities in women. The histopathology shows a proliferation of narrow vessels within thickened collagen bundles associated with multinucleate giant cells. These lesions are probably reactive in nature, and several mechanisms of pathogenesis, including hormonal, have been proposed. Different modalities, including intense pulsed light and pulsed-dye laser, have been used for treatment of these lesions. We report a case of a 74-year-old Caucasian woman with long-standing multinucleate angiohistiocytoma on her bilateral thighs that eluded diagnosis for several years. Upon biopsy and histopathological analysis, the diagnosis was made. Treatment options were entertained, although ultimately not pursued by the patient. We report this case to increase clinical awareness of this rare disease and to contribute to the ongoing literature aimed to further characterize this condition.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Multinucleate cell angiohistiocytoma: clinicopathological correlation of 142 cases with insights into etiology and pathogenesis.

Multinucleate cell angiohistiocytoma (MCAH) is a peculiar dermatopathological entity described as asymptomatic grouped red-to-violaceous papules, developing over weeks to months without spontaneous regression. The histopathological findings comprise bizarre basophilic multinucleated cells (MC), small vessel inflammation, mild dermal fibrosis, and a sparse lymphohistiocytic infiltrate. This study aimed to collate and analyze the clinical, histological, and immunohistochemical characteristics of all reported cases of MCAH from the international literature, and the presence or absence of concurrent chronic inflammatory or neoplastic phenomena to investigate any potential clinicopathological correlations, which may hint at the underlying pathophysiology of this condition. A systematic review of the literature was undertaken with information collected by a predeveloped pro forma. New case reports were also sourced from patient records at the Skin and Cancer Foundation Australia Database. A total of 142 cases of MCAH were collated, including 8 new case reports. The average age of onset was 50.1 years, with 79% of all individuals being female. The most commonly affected areas were the hands (30%) and face (29%). Univariate analysis revealed a positive association between lesion size and MC staining for CD68 (R = 0.488; P = 0.004), and an inverse relationship between size and endothelial staining for CD34 (R = -0.530; P = 0.012). Multiple lesions were significantly associated with an inverse relationship to MC staining of CD68 (R = -0.519; P = 0.002). Moderate correlations were seen between specific sites of vascularity and sites in which MCs were identified (R = 0.734-0.741; P < 0.001), and dermal fibrosis was associated with an increased number of MCs (R = 0.522; P = 0.002) and decreased multinucleate cell immunohistochemical staining (R = -0.655; P = 0.003). An association was found between patients with chronic inflammatory conditions and endothelial staining for CD68 (R = 0.671; P = 0.012), and an inverse relationship with MC staining for factor XIIIa (-0.481; P < 0.001). No statistically significant relationships between neoplasia and MCAH were found. From the data examined, we hypothesize that although this condition may be inflammatory and vascular in initial origin, fibrosis and atrophy play a vital role in the pathogenesis, particularly regarding the progression to multiple lesions. A detailed hypothesis is described that may be amenable to more detailed investigations. Limitations in this study include the heterogeneity of results analyzed across case reports; however, our conclusions match those developed through the analysis of our case series. These hypotheses and proposals provide an experimental basis for further research into the pathogenesis and mechanisms underlying MCAH.

Multinucleate cell angiohistiocytoma versus symplastic hemangioma - diagnostic dilemma

Symplastic hemangioma (SH) represents degenerative changes occurring in preexisting hemangioma that can mimic sarcomas and pseudosarcomas. Around eight cases of SH have been reported so far in the literature indicating its rare presentation. On the other hand, multinucleate cell angiohistiocytoma (MCAH) is believed to be a reactive inflammatory process rather than a true neoplastic process. Around 80 cases of MCAH have been reported so far. Both these entities share common histopathological features such as the presence of vascular channels, bizarre cells, inflammatory stroma, and multinucleate giant cells making them to masquerade sarcoma. Hereby, we report a case of a tiny swelling over the dorsum of hand in a 30-year-old female, with a clinical diagnosis of hemangioma. The histopathological examination revealed poorly circumscribed vascular lesion with bizarre cells, multinucleated giant cells scattered against the dense inflammatory background. We considered differential diagnoses of MCAH, SH, and dermatofibroma. We also considered malignancies in differential diagnoses such as hemangioendothelioma, liposarcoma, and metastatic nodule due to the presence of bizarre cells. This case report reemphasizes the presence of atypical histological features that represent degenerative changes occurring in benign tumors which can masquerade malignancies. We discuss the histological features that will help to differentiate SH and MCAH.

Multinucleate cell angiohistiocytoma: a case report and review of the literature.

Multinucleate cell angiohistiocytoma (MCAH) is a rare, benign vascular proliferation. Fewer than 80 cases have been reported to date, which may relate to under-recognition of this entity. Lesions are commonly asymptomatic and appear as erythematous to violaceous papules on the lower extremities and dorsal hands of middle-aged to elderly women. The characteristic histopathologic and immunohistochemical features of MCAH are essential for definitive diagnosis of MCAH. Multinucleate cell angiohistiocytoma follows a slowly progressive course, although spontaneous regression has been reported in rare cases. We present a case of MCAH to increase awareness and elucidate the characteristic clinical and histopathologic features of this disorder.

Everything you wanted to know about dermatofibroma but were afraid to ask

Everything you wanted to know about dermatofibroma but were afraid to ask

Multinucleate cell angiohistiocytoma with hypertrophic nerves

Multinucleate cell angiohistiocytoma (MCAH) represents a rare benign skin lesion characterized by multiple papules that are usually found on the distal extremities or face of middle-aged women. We report on a 60-year-old male with a history of monoclonal gammopathy and severe rheumatoid arthritis who had several asymptomatic red-to-livid papules grouped on the right side of his trunk. The lesions had been present for a few years and were gradually enlarging. Biopsies from three lesions showed a spectrum of changes consisting of a proliferation of small venules together with thickened collagen bundles and increased numbers of interstitial cells including bizarre, multinucleated giant cells. Immunohistochemically, the multinucleated cells were positive for vimentin and lysozyme while the interstitial cells expressed CD68, factor XIIIa and lysozyme. Interestingly, strikingly enlarged dermal nerves were seen in all the three biopsies and two of the biopsies showed an accompanying infiltrate of lymphocytes and plasma cells.

Nonepithelial skin tumors with multinucleated giant cells

Recognition of the different types of multinucleated giant cells in neoplastic and pseudotumoral lesions of the skin may be helpful in the differential diagnosis of these tumors. In this review, we will analyze the different types of multinucleated giant cells that can be found in nonepithelial cutaneous tumors and, more importantly, the clinicopathological context in which they are found. Touton giant cells are typically present in juvenile xanthogranuloma, necrobiotic xanthogranuloma, and some subtypes of xanthomas. Giant cells with a ground glass appearance are typically present in the solitary reticulohistiocytoma and multicentric reticulohistiocytosis. Osteoclast-like cells are found in giant cell tumors (GCT) of soft parts, plexiform fibrohistiocytic tumor, and atypical fibroxanthoma. Floret-like cells are present in giant cell fibroblastoma, pleomorphic lipoma, multinucleate cell angiohistiocytoma, and giant cell collagenoma.

Estrogen Receptor Alpha Overexpression In Multinucleate Cell Angiohistiocytoma: New Insights Into the Pathogenesis of a Reactive Process

Multinucleate cell angiohistiocytoma (MCAH) is a putative reactive skin lesion, which has been reported mostly in adult female patients, and hormones have been recently suggested as a factor influencing its development. To test this hypothesis, monoclonal anti-estrogen receptor (ER) a, anti-progesterone receptor (PR), anti-Factor XIIIa, and polyclonal anti-vascular endothelial growth factor (VEGF) antibodies were applied by immunohistochemistry on a series of 21 MCAHs and, compared to 10 cases of dermatofibroma (DF) and 5 cases of chronic dermatitis presenting multinucleate cells. On normal skin surrounding the lesions and in cases of DF and chronic dermatitis, ERa was expressed in few undifferentiated and differentiated sebocytes, in few cells of anagen follicles and perifollicular mesenchymal cells, and in scattered dermal fibrocytes. In MCAHs, ERa was strongly expressed in interstitial spindle cells, spindle cells surrounding the characteristic vascular component of the lesion, and rare multinucleate cells. PR expression was restricted to sparse spindle cells and few sebocytes, whereas Factor XIIIa stained spindle cells in the dermis of normal and inflamed skin and in both MCAH and DF, and VEGF staining resulted completely negative. ERα, PR, and VEGF have been reported as poorly expressed in normal skin. In the present study, expression of ERα resulted upregulated in cases of MCAH, particularly in spindle cells, compared with that in DF cases. These findings underline the pathogenetic difference between MCAH and DF and also corroborate the suggested hypothesis of a role played by hormones, mainly ERα, in the development of this lesion. These observations could open a new strategy in the treatment of MCAH by antiestrogen therapy.

Novel Case of Generalized Multinucleate Cell Angiohistiocytoma

multinucleate cell angiohistiocytoma is a rare benign fibrohistiocytic and vascular proliferation, typically characterized by the development of solitary papules, in an acral distribution in otherwise healthy late middle-aged to elderly women. our objectives are to present a novel case of generalized multinucleate cell angiohistiocytoma and to review the current literature regarding the clinical and histologic findings in this condition, as well as its potential causes and treatments. we describe a 35-year-old man who presented with generalized asymptomatic firm violaceous papules. Histopathology revealed dermal vascular proliferation; a perivascular infiltrate of lymphocytes, histiocytes, neutrophils, and plasma cells; thickened surrounding collagen bundles; and characteristic multinucleate cells with scalloped borders. our patient is one of three patients reported to date with generalized lesions of multinucleate cell angiohistiocytoma who were all in a younger age group (20-40 years old) than previously reported for solitary lesions.

Multinucleate cell angiohistiocytoma

Multinucleate cell angiohistiocytoma is a rare, benign, usually asymptomatic fibrohistiocytic and vascular proliferation which occurs predominantly on the extremities of middle-aged women. Histology shows characteristic multinucleate cells and is indispensable for diagnosis. We present a 42-year-old man from Egypt who was diagnosed with multinucleate cell angiohistiocytoma on the left cheek. As no malignant transformation has been reported, with the patient's agreement, no treatment was performed.

Multinucleate cell angiohistiocytoma: Report of three new cases and literature review

Multinucleate cell angiohistiocytoma (MCA) is a rare benign vascular proliferation of the skin of unknown cause. About 75 cases have been reported previously. We present herein three new cases of MCA studied immunohistologically and present a review the relevant literature that delineates the salient clinicopathological features of this unusual entity.