Incidence of mature adipocytic component within cutaneous smooth muscle neoplasms

Angioleiomyoma is a benign smooth muscle tumor of the subcutis. The presence of mature adipocytes has been described in this tumor under the rubric of ‘angiolipoleiomyoma’ or, erroneously, ‘angiomyolipoma’ (these are not PEComas). Previous studies have found adipocytes in only 2–3% of angioleiomyoma. Anecdotally, the incidence appeared to be greater than this in our practice. Moreover, the presence of adipocytes has not been evaluated in pilar leiomyoma or cutaneous leiomyosarcoma. We searched the pathology archives from 2007 to 2014 for all cutaneous and subcutaneous leiomyoma and leiomyosarcoma; cases were reviewed to confirm the diagnosis and evaluate for mature adipocytes. Seven of 73 total cases (10%) contained mature adipocytes: 1 of 33 pilar leiomyoma (3%), 4 of 22 angioleiomyoma (18%) and 2 of 18 leiomyosarcoma (11%). In our series, the 18% incidence of ‘angioleiomyoma with fat’ (our preferred terminology) is higher than the previously reported incidence of 2–3%. We also report the rare presence of mature adipocytes within pilar leiomyoma and cutaneous leiomyosarcoma, a finding not previously reported to our knowledge. Mature adipocytes may be present within cutaneous and subcutaneous leiomyomas and leiomyosarcomas and should not detract from the diagnosis or lead to concern for an adipocytic neoplasm or PEComa.     

Genital leiomyomas of the male nipple: two cases

BACKGROUND: Genital leiomyoma is a rare benign solitary skin tumor, not painful, developed from smooth muscle. Genital leiomyoma arising from the nipple is extremely rare, especially in males.CASES REPORT: A 47 year-old male had a 2 cm cutaneous plaque with nodules located on the right nipple. This plaque was circumscribed, erythematous, pruriginous and was not painful. The lesion had been noticed by the patient two years ago. A 37 year-old male showed a 1.5 cm cutaneous plaque located on the left nipple. The plaque was slightly erythematous, pruriginous, not painful and had been noticed by the patient 5 years earlier. Histology provided the diagnosis of genital leiomyoma in both cases. No surgical therapy was performed.DISCUSSION: Cutaneous leiomyomas are classified in 3 types regarding their origin: multiple or solitary piloleiomyoma, arising from arectores pilorum muscles, solitary genital leiomyoma, arising from the dartoic, vulvar, or mammillary muscles, and solitary angioleiomyoma, arising from the vein muscles. Clinically, genital leiomyoma is a 1 cm diameter solitary erythematous, firm nodule. According to many authors, genital leiomyoma is asymptomatic, but in the 2 patients, the lesions were pruriginous. Surgical excision is usually performed.     

Cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma: a rare occurrence and potential diagnostic pitfall

A case of cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma is described. The patient was a 60‐year‐old man who presented with a painless nodule on his chest. Thorough clinical examination did not reveal any evidence of tumor elsewhere. A punch biopsy was performed which showed a benign spindle cell neoplasm with focal cellular pleomorphism that had smooth muscle differentiation and was clearly originating from the arrector pili muscle, consistent with a cutaneous symplastic pilar leiomyoma. Immunohistochemical studies were performed and the tumor cells were strongly positive for smooth muscle actin (SMA) and desmin. Given the clinical information of a mass on the chest, clinical evaluation was recommended and a re‐excision was performed. Histologically, the re‐excision showed two distinct populations of cells. The upper portion of the tumor mirrored the initial biopsy; however, the base of the tumor showed hypercellular areas composed of spindle cells with marked pleomorphism and increased number of mitoses. The diagnosis of a cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma was given. This is the first report of this association of such occurrence reported in the literature. Fons ME, Bachhuber T, Plaza JA. Cutaneous leiomyosarcoma originating in a symplastic pilar leiomyoma: a rare occurrence and potential diagnostic pitfall.     

Multiple piloleiomyomas associated with solitary angioleiomyoma.

A 58 year-old male had reddish scattered papules strictly confined to the right side of his upper back and neck, right shoulder, and right upper arm. Additionally, he also noticed a subcutaneous nodule on his left thigh. Histopathological examinations revealed that the papules on the back, nape of the neck, shoulder, and arm were multiple piloleiomyomas, as shown by the proliferation of bundles of smooth muscles in the dermis. The subcutaneous nodule of the thigh was angioleiomyoma with a well-circumscribed lesion composed of smooth muscles and blood vessels. Ipsilaterality and segmentality of the distribution of the papules of piloleiomyomas and probable family history that his mother had similar papules with a similar distribution suggest the nevoid character of our case. Piloleiomyomas are often reported to be associated with leiomyoma of the uterus or other organs. This is the first reported case of multiple piloleiomyomas with solitary angioleiomyoma in the literature.     

Rapid Growing Superficial Cutaneous Leiomyosarcoma of the Face

Leiomyosarcomas are uncommon malignant smooth muscle tumors, mainly derived from vessels or viscera. Superficial leiomyosarcomas are a rare soft tissue sarcoma arising from the dermis or subcutaneous tissue in the skin. According to tumor origin and location, they are divided into cutaneous and subcutaneous leiomyosarcoma. They have distinctly different histologic and prognostic features from each other. Superficial leiomyosarcomas show a predilection for the proximal extremities and tend to be slow growing. We report one rare case of superficial cutaneous leiomyosarcoma on the right temporal area of face, which showed an extremely rapid growing mass within 3 months.     

Leiomyosarcoma of the lip

BACKGROUND: Superficial leiomyosarcomas are rare malignant tumors that may be subdivided into cutaneous and subcutaneous cases. CASE-REPORT: A 58 year-old man was seen for a lower lip tumor that appeared 3 years earlier. Histopathologic and immunohistochemical examinations showed features of cutaneous leiomyosarcoma. DISCUSSION: Cutaneous leiomyosarcoma may derive from the arrector pili, smooth muscle of sudoral glands or genital dartoic muscle. The subcutaneous form arises from smooth muscle wall of blood vessels. Superficial leiomyosarcomas occur frequently in the extensor surface of the lower extremities and involve the mucosal area in rare cases. Only five cases arising in the lips have been described. Lip cutaneous leiomyosarcoma may derive from ectopic sweat glands of the lips or from a hypodermic tumor that extends to the lip.     

Primary cutaneous carcinosarcoma arising in a patient with nevoid basal cell carcinoma syndrome

Background:  Nevoid basal cell carcinoma syndrome (NBCC) is an autosomal dominant disorder characterized by developmental abnormalities and neoplasms including basal cell carcinoma (BCC) and sarcomas (i.e. leiomyosarcoma, rhabdomyosarcoma, and fibrosarcoma). Primary cutaneous carcinosarcoma (PCC), a rare tumor composed of malignant epithelial and mesenchymal components, has never been previously described in association with this syndrome.
Case report:  A 61-year-old Hispanic man with a history of NBCC presented with a 4 cm nodule on the right proximal medial thigh.
Pathologic findings:  Areas of typical BCC merged with intersecting fascicles of large atypical spindle cells that stained for vimentin and were negative for actin, desmin, CD-34, and S-100 protein. Scattered bizarre solitary cytokeratin-positive epithelioid cells were embedded within the fibrocytic proliferation.
Conclusions:  Several carcinosarcomas have been reported to contain BCC as the malignant epithelial component, but to our knowledge, this is the first report of PCC associated with NBCC. Mutation in patched tumor suppressor gene on chromosome 9q occurs in BCCs of NBCC, and aberrancies on chromosome 9q are also reported in some carcinosarcomas. It is possible that the known genetic defect on chromosome 9 in this patient contributed to the development of carcinosarcoma.     

Superficial cutaneous leiomyosarcoma of the face: report of three cases

Leiomyosarcomas are rare malignant tumors of smooth muscles. Superficial leiomyosarcomas are classified into two subtypes depending on their location: cutaneous forms arise from the arrector pili muscles of hair follicles or dartos muscles of genital skin; subcutaneous forms arise from the smooth muscle lining of arterioles and veins in subcutaneous tissue. Superficial cutaneous leiomyosarcomas on the head are very rare. We report three cases of superficial cutaneous leiomyosarcoma, one localized on the face and two on the forehead.     

Primary cutaneous leiomyosarcoma

Primary leiomyosarcomas of the superficial soft tissues are very rare neoplasms. Because of the different biological behaviour, they should be divided into cutaneous and subcutaneous tumours. Differential diagnosis includes leiomyomas, skin metastases from extracutaneous leiomyosarcomas, and other spindle-cell tumours of the skin. Ultrastructural and immunohistochemical investigations are useful when light microscopic features do not allow an unequivocal diagnosis. Although local recurrences develop in almost half the patients with cutaneous leiomyosarcomas, the prognosis is predominantly good. In contrast, metastatic spread is more common in patients with subcutaneous leiomyosarcomas. Treatment for superficial leiomyosarcomas consists in wide excision. We report on a 76-year-old women with a local recurrence of a primary cutaneous leiomyosarcoma.     

Pleomorphic angioleiomyoma

We describe two cases of pleomorphic angioleiomyoma. In one case, a 46-year-old man presented with a single nodule on his scrotum of 1 year's duration, and in another, a 38-year-old woman presented with a single nodule on her right knee of 1 year's duration. In both cases, histopathologic examination showed a well-circumscribed nodule composed of smooth muscle and numerous veins and capillaries. Contrary to the ordinary angioleiomyoma, marked nuclear pleomorphism was noted. Although mitoses were rare, immunohistochemistry revealed many tumor cells that were positive for proliferating cell nuclear antigen, Ki-67, and p53, indicating that the pleomorphic appearance does not simply represent a degenerative change of some tumor cells.     

Congenital cutaneous angioleiomyoma

Congenital cutaneous angioleiomyoma is an extremely rare benign smooth muscle tumor. We present a case of a firm, painful subcutaneous mass noticed at birth on the left leg that on surgical excision proved to be an angioleiomyoma. Prognosis is good, and recurrences are uncommon. To our knowledge, this is the second report of a congenital angioleiomyoma.     

Myxoid Cellular Neurothekeoma: A New Entity of S100-Negative,CD68-Positive Myxoid Neurothekeoma

Neurothekeoma is a rare cutaneous neoplasm, occurring as a cutaneous papule or nodule on the face, shoulders, and upper extremities. Neurothekeoma has been subclassified as either the myxoid, cellular, or mixed type, depending on the amount of myxoid matrix and on immunohistochemical analysis. We observed a clinical case with conflicting histopathological and immunohistochemical findings. In this case, microscopic examination showed the typical presentation of myxoid neurothekeoma; however, immunohistochemical staining was negative for S100 protein and positive for CD68, which is the characteristic pattern of cellular neurothekeoma. We report a very rare form of myxoid cellular neurothekeoma of the face in a young woman.     

Cutaneous myxoid fibroblastoma. A histological, immunohistochemical, and ultrastructural study.

A fibroblastic skin tumor with a myxoid matrix is reported that cannot be easily classified as one of the well-known entities of fibrous/fibrohistiocytic and myxoid skin tumors. A 27-year-old white woman presented with a reddish, dome-shaped cutaneous nodule 8 mm in diameter on the left popliteal fossa that had developed spontaneously within the preceding 2 years. There was no sign of recurrence 30 months after excision. Light microscopic examination showed a well-circumscribed tumor confined to the upper dermis and consisting of stellate and spindle-shaped cells arranged loosely in a fascicular pattern resembling tissue cultures of fibroblasts. There were almost no collagen bundles between tumor cells, and Mowry's staining showed large amounts of glycosaminoglycans. Immunohistochemical studies of the tumor cells showed reactivity only to vimentin, whereas markers of histiocytes, dermal dendrocytes, and neurogenic and myogenic differentiation were negative. By electron microscopy, the majority of tumor cells contained elliptical nuclei, but some tumor cells had conspicuous multisegmented nuclei with several large and small nuclear segments connected by thin nuclear bridges (labyrinth nuclei). Single fibrils were found within the interstitium; collagen fibers were rare. Histological and ultrastructural examinations identified tumor cells as fibroblasts. High cellularity distinguishes this tumor from cutaneous myxoma. We conclude that this lesion represents a newly recognized tumor of fibroblastic origin. The name cutaneous myxoid fibroblastoma is proposed.     

Cutaneous Leiomyosarcoma in a Child

Abstract: Superficial leiomyosarcomas are infrequent tumors that are very rare in childhood. We report on a 12-year-old white boy with a cutaneous leiomyosarcoma of his left groin. Immunohistochemical study revealed positive immunostaining with antibodies to vimentin, desmin, and smooth muscle actin. The tumor was removed with wide surgical margins.     

Unusual clinical features of cutaneous tuberculosis in an immune compromised patient

We report a case of an unusual form of cutaneous tuberculosis in an 82-year-old woman. She visited our hospital because of an intractable ulcer on the fifth finger of her right hand. While examining the ulcer surrounding half of her right fifth finger and covered with necrotic tissue, we also perceived a nodule with crust on the forearm, multiple subcutaneous nodules on the right forearm and upper arm, and a hen's-egg-sized agglomerative nodule on the axilla. All the lesions were located on her right arm. Skin biopsy specimens showed granulomatous tissue with necrosis in the lesions. Mycobacterium tuberculosis was identified by culture of a biopsied specimen, so the diagnosis was confirmed. Further examination revealed that she also had pulmonary tuberculosis. Cutaneous tuberculoses are classified morphologically with reference to host immune status, but no satisfactory classification exists. The present case can't be classified into any of the types which have been proposed so far. She is elderly and suffers from liver cirrhosis, hepatocellular carcinoma and myelodysplastic syndrome. The resulting acquired immunosuppression may have caused a unique form of cutaneous tuberculosis.     

Giant cutaneous melanoacanthoma

A 45‐year‐old male cook was seen for the evaluation and removal of a “mole on my eye” of steady growth during the previous 6 months. The patient stated that he had had a “brown spot” above his left eye for 17 years prior to its recent enlargement; it was now partly blocking the vision in his left eye. He denied a history or family history of cutaneous tumors, including skin cancer. On examination, a 2.5‐cm × 0.5‐cm × 0.8‐cm, horn‐like, darkly pigmented, cutaneous nodule was evident extending from the left upper eyelid downwards to below the lower eyelid ( Figs 1 and 2 ). He also had scattered, skin‐colored, 2–3‐mm cystic papules on his anterior mid‐chest. A shave excision specimen was obtained from the eyelid nodule.

Figure 1 Open in figure viewer PowerPoint Horn‐like, cutaneous nodule extending from the left upper eyelid downwards to below the lower eyelid     

Malignant giant cell tumor of the tendon sheath

A 27‐year‐old man presented with a slow‐growing, painless mass on the right anterior forearm of 4 years’ duration. Because of apparent rapid enlargement of the mass, he underwent surgical excision in a local clinic without histopathologic examination in June 1999. Recurrence of the tumor was noted in August 1999, when an excisional biopsy was performed. The pathologic diagnosis was giant cell tumor of the tendon sheath. In December 1999, he presented to our dermatology clinic with a local recurrence at the same site. He denied any history of trauma or any particular event preceding the initial appearance of the mass. On physical examination, there was a skin‐colored subcutaneous nodule, about 1.5 cm in size, under the operative scar on the right anterior forearm ( Fig. 1 ). We performed an excisional biopsy. The pathology showed features of osteosarcoma. No bony involvement was seen on X‐ray or bone scan. Routine blood chemistry and blood count were normal.

Figure 1 Open in figure viewer PowerPoint A subcutaneous nodule under the operative scar on the right forearm     

Silver deposits in cutaneous burn scar tissue is a common phenomenon following application of a silver dressing

Background:  Silver dressings have been widely and successfully used to prevent cutaneous wounds, including burns, chronic ulcers, dermatitis and other cutaneous conditions, from infection. However, in a few cases, skin discolouration or argyria-like appearances have been reported. This study investigated the level of silver in scar tissue post-burn injury following application of Acticoat™, a silver dressing.
Methods:  A porcine deep dermal partial thickness burn model was used. Burn wounds were treated with this silver dressing until completion of re-epithelialization, and silver levels were measured in a total of 160 scars and normal tissues.
Results:  The mean level of silver in scar tissue covered with silver dressings was 136 μg/g, while the silver level in normal skin was less than 0.747 μg/g. A number of wounds had a slate-grey appearance, and dissection of the scars revealed brown-black pigment mostly in the middle and deep dermis within the scar. The level of silver and the severity of the slate-grey discolouration were correlated with the length of time of the silver dressing application.
Conclusions:  These results show that silver deposition in cutaneous scar tissue is a common phenomenon, and higher levels of silver deposits and severe skin discolouration are correlated with an increase in the duration of this silver dressing application.     

流脑疫苗接种后毛母质瘤1例

患者女,6岁,右上臂外侧肿物半年余。皮肤科情况:右上臂外侧见一约5.0 cm×2.0 cm×1.0 cm大小椭圆形淡红色肿物,边界清楚,质稍硬,可推动,无触痛。皮损组织病理示:基底样细胞增生并过渡为影细胞,伴异物肉芽肿形成及炎细胞浸润。诊断:毛母质瘤。