Cutaneous metastases from Ewing's sarcoma: report of two cases

Ewing's sarcoma is a malignant osseous neoplasm that affects mostly children and young adult males. Clinically, the neoplasm presents with oedema, swelling, and pain of the involved area. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells, with vesicular nuclei and scant cytoplasm, arranged in irregular masses separated by strands of fibrous tissue, with areas of necrosis en masse intermingled with intratumoural haemorrhage. Ewing's sarcoma is an extremely aggressive neoplasm and metastases to sites such as lung, pleura, other bones, central nervous system, liver, and regional lymph nodes frequently develop in early stages of the disease. Surprisingly, despite the highly aggressive biological behaviour of this neoplasm, cutaneous metastases from Ewing's sarcoma are very uncommon. We report two patients with Ewing's sarcoma of the bone who developed cutaneous metastases. As in other internal malignancies, the onset of cutaneous metastases in patients with Ewing's sarcoma indicates a poor prognosis.     

不同皮疹类型的结节病三例

<正>结节病是一个多系统受累并以裸结节为病理表现的一类疾病[1]。其病因不明,通常涉及多系统损害,包括肺、淋巴结、关节、皮肤及眼等[2]。本病的自然病程多样,可以从无症状到突发猝死[2]。大约25%～35%的系统性结节病患者有皮肤的受累[3]。结节病的皮肤表现多样,常导致临床的误诊[4]。此外,不同的皮疹表现和系统受累严重程度之间的关系目前尚不清楚,而且结节病的治疗通常比较棘手[5]。我们报道3例以不同皮疹类型为表现的系统性结节病。临     

Epithelioid sarcoma metastatic to the tongue: a rare entity

Epithelioid sarcoma (ES) typically arises as a firm nodule on the extremities of young men. The tumor is remarkable for diagnostic difficulties both clinically and histopathologically resulting in a high frequency of initial misdiagnosis. ES is also known to have a high rate of recurrence and high rate of metastasis predominantly to the lymph nodes, lungs, and scalp. Herein, the second case of ES with metastasis to the tongue is reported.     

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.     

Unresponsive cutaneous leishmaniasis and HIV co-infection: report of three cases

Cutaneous leishmaniasis (CL) is a vector borne disease caused by various species of Leishmania parasite. CL is endemic in the Thar desert of Rajasthan state and Himachal Pradesh in India. Immune suppression caused by human immunodeficiency virus (HIV) infection is associated with atypical clinical presentation of CL which responds poorly to the standard treatment and causes frequent relapses. We are reporting three cases of localized and disseminated CL due to Leishmania tropica which failed to respond to conventional intralesional/intramuscular sodium stibogluconate (SSG) injections. Initially, we did not think of HIV infection because CL is endemic in this region. When patients did not respond to SSG injections, we performed enzyme-linked immunosorbent assay (ELISA) tests for HIV and they turned out to be HIV positive. Our report showed that CL is emerging as an opportunistic infection associated with HIV/AIDS and may be the first manifestation in HIV positive patients in an endemic area.     

Segmental congenital hemangiomas: Three cases of a rare entity

Congenital hemangiomas (CHs) are unusual and diverse tumors distinguished from infantile hemangiomas by being largely developed at birth and glucose transporter (GLUT1)-negative. We describe three infants who presented in utero or at birth with segmentally distributed vascular tumors that were GLUT1-negative, had histology compatible with congenital hemangioma, and exhibited spontaneous clinical involution. One of the three patients had high-output cardiac failure and was found to have a mutation in GNAQ (c.626A>c, p.Gln209Pro); another had high-output cardiac failure, heterotaxy, and transient hematologic abnormalities and was found to have a mutation in GNA11 (c.626_627delinsCC, p.Gln209Pro). In addition to describing a novel segmental pattern of congenital hemangioma variant with genetic correlations, these cases illustrate the utility of targeted genetic testing to elucidate the exact mutation and thus classification of vascular tumors.     

Eccrine squamous syringometaplasia secondary to cutaneous extravasation of docetaxel: report of three cases

Eccrine squamous syringometaplasia is characterized by the metaplasia of cuboidal epithelial cells of the eccrine sweat ducts into squamous epithelial cells. It has been associated with several conditions including chemotherapy‐related bilateral dermatitis, an entity that can take place in body areas rich in eccrine glands, as well as in acral erythema related to chemotherapy. Only a few cases because of cutaneous extravasation of chemotherapy have been previously reported. We report three cases of eccrine squamous syringometaplasia secondary to extravasation of docetaxel.     

Primary cutaneous epithelioid rhabdomyosarcoma: a rare,recently described entity with review of the literature

We report a case of a primary cutaneous epithelioid rhabdomyosarcoma that presented as a single raised pink‐purple lesion (3.3 × 2.2 cm) on the left base of neck in a 75‐year‐old man. Histopathologic examination revealed an exophytic, nodular tumor within the dermis and superficial subcutis with overlying ulceration. The tumor exhibited sheet‐like growth, infiltration of adjacent structures, and was composed of uniform epithelioid cells with abundant eosinophilic cytoplasm and eccentrically placed vesicular nuclei with irregular nuclear contours and prominent central nucleoli. Numerous mitotic figures were present [28/10 high power fields (HPF)] but only mild cytologic pleomorphism was identified. By immunohistochemistry, tumor cells were diffusely and strongly positive for desmin and MYOD1. Focal positive staining for myogenin and cytokeratin CK903 was identified. Stains for Melan‐A, S‐100, SOX10, p63 and CK5/6 were negative. These histopathologic and immunophenotypic features support a diagnosis of epithelioid rhabdomyosarcoma. No evidence of a deep soft tissue primary lesion was identified. In summary, epithelioid rhabdomyosarcoma can present as a primary cutaneous lesion and dermatopathologists should be aware of this entity.     

Donovanosis: report of two cases with rare complications

2 cases of Donovanosis with unusual presentations, anal stricture in a 25-year old man, and massive enlargement of the labia in a 25-year old woman, are described. The man had a 15 x 6 cm laque from the natal cleft to the scrotum, constricting the anus so that he could only pass painful ribbon stools. The center was ulcerated for 4 cm around the anus. The patient had no other positive laboratory or clinical findings. The lesion had developed from a pustule that developed into an ulcerative lesion 7 years ago, just after a homosexual contact. A tissue smear, but no a biopsy, showed Donovan bodies. The patient was cured with 21 days of oral sulfamethoxazole-trimethoprim, 2 tablets of 400/80 mg twice daily. The woman's labia were both solidly enlarged and edematous, surrounded by ulceration of the groins and perineum. She also had no positive laboratory or systemic clinical findings. Her biopsy showed a few Donovan bodies, but repeated tissue smears were negative. Her ulcer was healed completely in 20 days with oral tetracycline, 2.0 g daily, but her labia remained swollen.     

Expanding the differential of superficial tumors with round-cell morphology: Report of three cases of CIC-rearranged sarcoma,a potentially under-recognized entity

Among sarcomas with a round-cell morphology that lack rearrangement of the EWSR1 gene, rearrangements involving the CIC gene are the most common. In comparison with Ewing Sarcoma, CIC-rearranged sarcomas present at an older average age, arise almost exclusively in soft tissues, are clinically more aggressive, and are more likely to be resistant to the chemotherapy regimens used for Ewing sarcoma. CIC-rearranged sarcomas present more commonly in a deep location, and we suspect that superficial presentations may be under-recognized. In this case series, we report three of such cases. Overall, the morphology is similar to CIC-rearranged sarcomas of deeper locations. We hope to raise awareness among the dermatopathology community by expanding the differential of superficial tumors with round cell morphology.     

Melanocytic matricoma: two cases of a rare entity in women

Melanocytic matricoma is a rare cutaneous adnexal tumor occurring in humans with only 13 cases reported in the literature. The typical lesion is a circumscribed pigmented nodule on sun‐damaged skin. Most cases have occurred in elderly men. The tumor contains a mixed population of matrical cells, supramatrical cells, shadow or ghost cells, and dendritic melanocytes. We report two cases of melanocytic matricoma in two elderly women with unusual histopathological features such as cystic degeneration and focal granulomatous inflammation, which are considered to be atypical for this entity.     

Giant proliferating pilomatricoma; report of a rare entity

Proliferating pilomatricoma is a benign tumour and a rare variant of pilomatricoma that has the potential for local recurrence if incompletely excised. We report a case of giant proliferating pilomatricoma on the forearm of a 66‐year‐old woman. This tumour was unusually large and the presence of ulceration and rapid growth raised clinical suspicion of malignancy. The identification of shadow or ghost cells is a good clue to matrical differentiation, which can be confirmed by β‐catenin immunostaining.     

皮肤异色病样淀粉样变病二例

患者1,男,49岁,病程9年;患者2,女,21岁,病程3年。两例患者均表现为全身弥漫性色素沉着夹杂点状白斑,伴苔藓样丘疹,偶有水疱。家族中无类似病史。组织病理示：真皮乳头层淀粉样物质沉积。     

Dermatoscopic features of cutaneous atypical fibroxanthoma: three cases

Atypical fibroxanthoma (AFX) is an uncommon, low‐grade, malignant, spindle‐cell tumour of fibrohistiocytic histogenesis, which can mimic other malignant skin tumours, such as basal and squamous cell carcinoma (CC), melanoma, and Merkel cell carcinoma (MCC). Three cases of AFX were examined by dermatoscopy, which revealed white areas and an atypical polymorphous vascular pattern characterized by the concurrence of different structures: linear, dotted, hairpin, arborescent and highly tortuous vessels, irregularly distributed over the surface. Seborrhoeic elements and photoageing may be accompanying features depending on the anatomical location of the AFX. AFX may be added to the list of slightly pigmented, reddish, malignant cutaneous tumours, such as SCC, MCC, amelanotic/hypomelanotic melanoma and eccrine porocarcinoma, which display prominent and chaotic dermatoscopic neoangiogenetic features in more advanced stages of proliferation.     

Primary cutaneous phaeohyphomycosis: report of seven cases

We report seven cases of primary cutaneous phaeohyphomycosis. There were five males and two females, ranging in age from 42–65 years (mean 57.7 years). Two patients were otherwise healthy, but five were immunocompromised. One patient had rheumatoid arthritis and was on oral prednisone; two were renal transplant recipients, one was a heart transplant recipient, and the fifth had dermatomyositis. No history of trauma was elicited from any of the patients, but in two cases, foreign material was seen in the tissue sections. All lesions were oil the extremities. In two cases, tissues were cultured, and these grew Exophiala jean-selmei . The others were not cultured because fungal infection was not clinically suspected. No systemic disease developed in any of the cases, and all were cured by the simple, complete excision of the lesions.