A case of radiation‐induced osteosarcoma of the skull presenting as a cutaneous epidermotropic tumor with a short latent period

Radiation‐induced sarcoma (RIS) is an unusual but well documented tumor. The frequency of RIS of the head and neck region has been reported as 0.143%. In the literature the median interval between irradiation and development of sarcoma is 11 years. Cases of RIS with a short latent period, that is, less than 4 years are rare. We report a case of a 34‐year‐old female who developed an osteosarcoma of the scalp, over a previous craniotomy scar, 3 years after excision of a frontal anaplastic oligodendroglioma which had been followed by a course of 6 weeks radiotherapy (58 Gy) and 6 cycles of temozolomide. The histological features were those of a high‐grade osteosarcoma with epidermotropism of tumor cells. Lymph nodes were partially replaced by high‐grade metastatic osteosarcoma, with extra‐nodal lymphatic tumor thrombi. To our knowledge the only other case report of post‐radiation osteosarcoma with a short latency period was a case of osteosarcoma in the craniofacial bone 3 years after radiotherapy for maxillary squamous cell carcinoma. The histological finding of prominent replacement of the epidermis by osteosarcoma has not been reported before.     

Chondroid syringoma of the arm: an unusual localization

Chondroid syringoma (CS) is an uncommon, benign, sweat-gland tumor. The usual presentation is of an asymptomatic, slowly growing mass, typically located on the head and neck region. We present a case of a CS located in the right arm of a 43-year-old woman. This tumor developed slowly over 8 years. The diagnosis of CS was made by histopathological examination; it showed an abundant chondroid stroma intermingled with a fibro-adipoid tissue. There was no recurrence after total excision of the tumor with a 2-year followup. The unusual localization of chondroid syringoma made an accurate preoperative diagnosis difficult and diagnosis was achieved only by microscopy. Surgical tumor excision remains the best therapeutic option to avoid relapses of this tumor. Close followup is recommended because malignant transformation, although rare, is possible.     

Primary mucinous carcinoma of the scalp

Primary cutaneous mucinous carcinoma (MC) is a rare epithelial neoplasm derived from the sweat glands. Herein, we report a case of MC located on the head. A 66-year-old woman underwent excision of a nodular tumor with a reddish brown surface on the left parietal region. Histopathology revealed a neoplasm extending from the reticular dermis into the subcutaneous fat. The tumor cell aggregates showed cribriform and solid lobules and were embedded in lakes of mucin, separated by thin, fibrous septae. Focally single neoplastic cells were arranged in an Indian-file pattern. The tumor cells displayed an eosinophilic cytoplasm, large basophilic nuclei and some discrete nuclear atypia. Vascular spaces, filled by densely packed erythrocytes between the septae, were also observed. We compared the mucinous component with the tumor cell and the stromal component by light microscopy. Analyzing the tumor by an image analysis system in Alcian-blue-stained serial sections, we found the averaged total tumor area measuring 99.7 mm(2). The area of the mucinous component measured 92.4 mm(2), that of the tumor cells 3.7 mm(2) and that of the stromal component 3.6 mm(2). The extensive checkup of the patient disclosed no evidence for a further malignant neoplasm. After excision of the tumor an adjuvant radiotherapy was performed. The patient was free of recurrence and metastatic spread of the mucinous carcinoma during a 4-year follow-up.     

Cutaneous myoepithelial neoplasms: clinicopathologic and immunohistochemical study of 20 cases suggesting a continuous spectrum ranging from benign mixed tumor of the skin to cutaneous myoepithelioma and myoepithelial carcinoma

BACKGROUND: Myoepithelial neoplasms, both benign and malignant, are rare but well-established clinicopathologic entities in the salivary glands, the breast, and the lung. Despite similarities between cutaneous sweat glands and glandular structures in the above-mentioned organs as well as the presence of regular myoepithelial cells around cutaneous eccrine/apocrine glands, the concept of cutaneous myoepithelial neoplasms is still debatable and not commonly accepted. METHODS: Twenty cutaneous myoepithelial neoplasms have been studied histologically and immunohistochemically. RESULTS: Nine neoplasms showed features of benign mixed tumor of the skin (chondroid syringoma) (five females and four males, age range 19-65 years, all cases arose in the head and neck region). Two cases represented the eccrine and seven the apocrine subtype. Interestingly, in three cases of the apocrine subtype, solid areas composed predominantly of myoepithelial cells were detected; these neoplasms were designated as benign mixed tumors with prominent myoepithelial cells. Nine cutaneous neoplasms were composed of spindled, epithelioid, and plasmocytoid cells without ductal differentiation and immunohistochemically stained variably positive for vimentin, epithelial and myogenic markers, S-100 protein, calponin, and glial fibrillary acidic protein (four females and five males, age range 3-71 years, four cases arose in the head and neck region and one case each on the finger, the thigh, the lower leg, the foot, and the breast, respectively); these neoplasms were designated as cutaneous myoepitheliomas. Two morphologically malignant neoplasms with cytologic and immunohistochemical features of myoepithelial cells arose on the face of a 70-year-old female and a 79-year-old male patient; these neoplasms were designated as malignant cutaneous myoepitheliomas (cutaneous myoepithelial carcinomas). CONCLUSIONS: The study suggests a continuous spectrum of cutaneous myoepithelial neoplasms ranging from benign mixed tumor of the skin to cutaneous myoepithelioma and cutaneous myoepithelial carcinoma. Further studies with extended follow-up information are necessary to establish prognostic factors.     

Multiple cellular neurothekeomas--a case report and review on the role of immunohistochemistry as a histologic adjunct

BACKGROUND: Cellular neurothekeoma is a relatively rare, benign cutaneous neoplasm, which usually presents as a solitary papule or nodule involving the head and neck area of young adults. Multiple neurothekeomas have not, to date, been known to occur. METHODS: We report a 30-year-old, otherwise healthy, male who presented with multiple neurothekeomas (15) in the head and neck area over a period of 12 years. RESULTS: While the unifying feature of all biopsied (10 of 15) lesions was the presence of epithelioid cells--the lesions differed in their cellularity and the degree of sclerosis of the stromal component. Antigenic profiling of the lesional cells revealed expression of vimentin, NKI/C3, PGP 9.5, factor XIIIa and CD68 but not S100, HMB45, MelanA, EMA, MSA, desmin, CD57 or NGF-R. CONCLUSIONS: This case report is the first to document the occurrence of multiple cellular neurothekeomas. An unusual histologic feature of some of the biopsied lesions was the presence of a markedly sclerotic stroma.     

Syringocystadenoma papilliferum in an unusual location

Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.     

Syringocystadenoma papilliferum in an unusual location beyond the head and neck region: a case report and review of literature

A case of syringocystadenoma papilliferum with multiple papulonodules in a linear fashion located in an unusual location of the right lower abdomen is presented. The presence of a large tumor at the inferior pole raised the suspicion of malignant transformation and the presence of discharge from the lesions raised the possibility of necrosis. However, histopathological examination showed the classical features of syringocystadenoma papilliferum without malignant transformation or tumor necrosis. The patient refused to undergo surgical excision of the nodules and subsequently was lost to follow-up. This case illustrates the atypical location of a rare disease and adds to the differential diagnosis of linear verrucous lesions on the abdomen. Review of all the cases with syringocystadenoma papilliferum outside the head and neck region in the English literature showed only one case of syringocystadenoma papilliferum arising on the abdomen; our patient is the second reported case with the unique feature of linear arrangement of lesions.     

Hyaline cell-rich chondroid syringoma of the finger

A mixed tumor is a neoplasm that has microscopic features of both epithelial and mesenchymal differentiation. Such mixed tumors are known as pleomorphic adenomas in the salivary glands, and their cutaneous counterparts are called chondroid syringomas. These tumors commonly occur in the head and neck region of middle-aged men. Hyaline cell-rich chondroid syringoma is a rare benign variant of chondroid syringoma composed of cells with eosinophilic hyaline cytoplasm and plasmacytoid features, the origin of which remains elusive. Although very few cases have been reported in literature, it is important to be aware of this entity so as to avoid misdiagnosis on histopathological examination. In this report we present a case of hyaline cell-rich chondroid syringoma occurring in the finger.     

Lipomatous mixed tumor of the skin

An unusual tumor of the neck in a 56-year-old female is reported. The tumor was composed of tubules and small cords of epithelial cells dispersed in the fibromyxoid and adipose stroma. At the periphery, the tumor was not encapsulated and its border was intermingled with the subcutaneous fat. Lack of decapitation secretion and the absence of long tubules suggested an eccrine origin; however, in some of the tumor areas, the cells showed brightly eosinophilic copious cytoplasm that may indicate an apocrine differentiation. As an area of chondroid metaplasia was identified, the diagnosis of a mixed tumor was rendered. This unusual type of skin adnexal neoplasm with unique relation of the epithelial component to the surrounding adipose tissue requires differentiation with the primary cutaneous and metastatic carcinomas.     

Cutaneous mixed tumor, eccrine variant: a clinicopathologic and immunohistochemical study of 50 cases, with emphasis on unusual histopathologic features

Mixed tumor, eccrine type, is a rare cutaneous adnexal neoplasm, mostly reported as isolated case reports. A systematic analysis of its histopathologic and immunohistochemical features has not previously been performed on a large series. The purpose of our investigation was to study a large number of cutaneous eccrine mixed tumors so as to fully characterize the entire spectrum of changes in the epithelial and stromal components, with an emphasis on unusual histopathologic features that may represent a diagnostic pitfall. This article reports a light microscopic and immunohistochemical study of 50 cases of eccrine mixed tumor, complemented by a literature review. Our study identified some unusual histopathologic features, thus extending the morphologic spectrum of this neoplasm. These included prominent cribriform areas, clear cell change, pseudorosette structures, prominent osseous metaplasia, and physaliphorous-like cells. Most of these features have not been previously recorded in eccrine mixed tumors and may represent a potential diagnostic pitfall.     

A red cheek as first clinical sign of a sebaceous lymphadenocarcinoma of the parotid gland with lymphangiosis carcinomatosa and lymph node metastases

Sebaceous lymphadenoma is a rare benign neoplasm presenting predominantly in the parotid gland or in periparotid lymph nodes. It rarely transforms into a malignant tumor. We present a sebaceous lymphadenoma with an unusual clinical presentation with a malignant component, infiltration of lymph nodes, and lymphangiosis carcinomatosa. Sebaceous lymphadenoma usually presents as a well-circumscribed, painless, infraauricular mass. In our 87-years-old male patient, the clinical presentation was confluent reddish livid plaques at the left cheek and the left neck. In addition, cervical lymph node metastases were suspected. After skin biopsy, the tumor was excised by parotidectomy, skin excision, and neck dissection followed by a postoperative radiotherapy. To our knowledge, this is the sixth reported case of sebaceous lymphadenocarcinoma and the first case presenting with cervical lymph node metastases and dermatological symptoms revealing lymphangiosis carcinomatosa.     

Eccrine Nevus on the Neck: A Case Report and Review of the Literature

Eccrine nevus is an extremely rare benign cutaneous hamartoma that usually occurs in childhood and adolescence. It has a wide range of clinical manifestations, and histological findings reveal a proliferation of structurally normal eccrine ducts. Herein, we present a case of eccrine nevus on the neck of an 8-year-old girl. Our literature review reveals that the head and neck region is a rare anatomical location for eccrine nevus as it has a predilection for extremities. Our review also suggests that overlying skin changes are common in eccrine nevus regardless of accompanying localized hyperhidrosis.     

Stromal cells as the major source for matrix metalloproteinase-2 in cutaneous melanoma

Matrix metalloproteinases (MMPs) are essential for tumor progression, invasion and metastases formation. Expression of these proteinases is not only restricted to the tumor cells themselves, but also is found in normal stromal cells. Moreover, immunohistochemistry suggests stromal cells as the major source. To scrutinize this hypothesis we established a slowly growing, syngeneic tumor model using the B16-melanoma cell line B78D14. In vitro analysis demonstrated that B78D14 cells secreted MMP-2, MT1-MMP, and to a lesser degree MMP-9; in addition they expressed both MT1-MMP and EMMPRIN on their surface. In subcutaneous (s.c.) tumors of these cells MMP-2 expression was predominantly present at the tumor-stroma border indicating stromal cells as primary source for this protease in vivo. Indeed, double staining experiments and in situ zymography confirmed that tumor adjacent stromal cells at the invasive front expressed MMP-2 and only at this site activated MMP-2 was detectable. Notably, in an experimental pulmonary metastases model neither tumor nor stromal cells expressed MMP-2, suggesting that the capacity of stromal cells is largely dependent on the surrounding microenvironment.     

Atypical cellular neurothekeoma: A potential diagnostic pitfall for benign and malignant spindle cell lesions in skin

Cellular neurothekeomas (CNTs) are rare, benign cutaneous tumors that arise primarily on the head and neck, with a slight female predominance. CNTs with atypical features have been described, including those with an infiltrative growth pattern. Although CNTs with atypical features are benign, recognition of this entity can pose diagnostic challenges. Here, we report a case of CNT with an unusual clinical presentation on the left second digit, and with atypical histological features including an infiltrative growth pattern, which could have been mistaken for features of malignancy.     

Dermal nodular fasciitis: three case reports of the head and neck and literature review

Nodular fasciitis is a benign reactive myofibroblastic proliferative process of unknown etiology. It presents as a solitary painless, rapidly growing nodule over several weeks' duration. The condition is self-limited, and proper diagnosis is essential to avoid unnecessary aggressive treatment. Diagnosis is often a challenge because it may be confused with a malignant tumor due to its aggressive clinical behavior and histological features. Immunohistochemical staining can be a useful tool to aid in the diagnosis. Although most commonly located on the extremities and then the trunk, it is estimated that the head and neck region represents only 10 to 20%. The majority of cases arise in the soft tissue, i.e. fascia, muscle, or subcutaneous tissue. Interestingly, cases in the head and neck region often involve dermal tissue. There have been five separate reports documenting rare cases of dermal nodular fasciitis in the dermatopathology literature and one case series involving 28 of 50 dermal variants from the external ear region. We report three additional cases of dermal nodular fasciitis occurring on the left cheek, base of the scalp, and right medial canthus.     

Angiosarcoma of the abdominal wall after dermolipectomy in a morbidly obese man. A rare form of presentation of Stewart-Treves syndrome

Angiosarcoma is a rare malignant tumor, with a predilection for skin in the head and neck region, although it has been described in many other locations. Its association with chronic lymphedema is well known, mainly in the setting of postmastectomy lymphedema of the arm in breast cancer patients (termed Stewart-Treves syndrome). However, angiosarcoma can appear in lower limbs with chronic lymphedema and rarely in other locations such as the abdominal wall. Herein, we present a unique case of angiosarcoma developing in the abdominal wall of a morbidly obese patient after extensive dermolipectomy.     

Folliculosebaceous cystic hamartoma of the nipple: a case report

Folliculosebaceous cystic hamartoma (FCH) is a relatively rare and recently described lesion of the skin. Mostly, it involves the skin of the head and neck area. It is considered by most authors as a form of non-neoplastic cutaneous hamartoma comprising follicular, sebaceous and stromal elements. However, few regard it as a sebaceous trichofolliculoma, i.e. a variant of trichofolliculoma. FCH of the nipple of female breast is a very rare lesion with only one previous case reported so far in the literature. We report a similar case with a brief review of the literature.     

Delayed diagnosis in a case of lupus vulgaris with unusual localization

Lupus vulgaris is the most common form of cutaneous tuberculosis, and the usual sites of involvement are the head and neck. We present a forty-six-year-old woman with lupus vulgaris on the external surface of the left leg and foot, an unusual site. Based on histopathological and clinical features, this case was diagnosed as lupus vulgaris with unusual localization.     

Basal cell carcinoma with a sarcomatous component (carcinosarcoma): a series of 5 cases and a review of the literature

Cutaneous carcinosarcomas are rare biphasic malignant tumors with a malignant epithelial component together with malignant stroma. Five cases treated in the Yorkshire region of England between 2003 and 2006 are presented. The patients were male with an age range from 58 to 84 years. Four lesions occurred on the face and one on the trunk. Each tumor had an epithelial component resembling a typical nodular basal cell carcinoma. The stromal components demonstrated atypical spindle cells and tumor giant cells, undifferentiated stroma, or osteoid formation. All underwent surgical excision and none showed evidence of recurrence (follow-up 3-17 months, one death from unrelated causes). The recent literature concerning the pathogenesis and prognosis of these unusual tumors is reviewed.     

Subcutaneous ossifying lipoma: case report and review of the literature

BACKGROUND: Lipomas can undergo a variety of changes, one of which is ossification. Ossifying lipoma (OL), independent of bone tissue, is rare. No case has been reported with this heading in the dermatopathological literature. CASE REPORT: A 73-year-old woman was referred for a subcutaneous mass on the left scapular region that had been present for more than 22 years. RESULTS: The lesion was an OL measuring 8 cm and weighing 75 g. Only 25 cases of OL have been described in soft tissues. Including the present report, locations in order of frequency are the head and neck area (11 cases), the limbs (9 cases) and the trunk (6 cases). Average patient age is 50.9 years (range, 17-81 years). History is measured in years (mean, more than 6 years). Average tumor size is 7.1 cm (range, 0.8-20 cm). The adipose component is usually predominant and the mature bone tissue is irregular in distribution. Bone spicules are surrounded by fibrous tissue bands. Chondrous structures are found in 46.2% of tumors. No recurrences have been reported. CONCLUSIONS: OL is rare but has a characteristic gross, radiological and histological appearance. It has the same prognosis as simple lipoma. Excision is the treatment to be chosen.