Epithelioid sarcoma with angiomatoid features: report of an unusual case arising in an elderly patient within a burn scar

Epithelioid sarcoma (ES) is a rare, aggressive soft tissue tumor with a characteristic predilection for adolescents and young adults, and a tendency to occur on distal extremities. We report a case of ES arising in an 80-year-old woman within a burn scar that histopathologically showed unusual 'angiomatoid' features. The patient presented initially with a solitary nodule on her right wrist arising at the site of a burn scar. Histopathologically, the tumor was composed of a proliferation of relatively bland, epithelioid and spindle cells focally arranged in a nodular pattern around areas of 'geographic' necrosis. In addition, there were prominent foci of hemorrhage and blood-filled spaces as well as tumor cells with intracytoplasmic vacuoles, features suggestive of an angiomatous process. Immunohistochemistry showed positivity of tumor cells for cytokeratins and epithelial membrane antigen (EMA) whereas all vascular markers tested were negative. The overall histopathologic features were consistent with a diagnosis of ES. Follow up showed multiple recurrences arising proximally along the right upper extremity. Our case underlines the clinical and histopathological heterogeneity of ES, emphasizing the unusual occurrence of ES with 'angiomatoid' features in the elderly. In this uncommon setting, this tumor should be especially distinguished from epithelioid hemangioendothelioma and epithelioid angiosarcoma. The significance of development of ES on a healed burn scar is uncertain, but may suggest a possible causal relationship.     

Metastatic rhabdoid melanoma: report of a case with a comparative review of the literature

Melanoma has a propensity to mimic the morphologic appearances of a wide variety of epithelial and soft tissue neoplasms. Although rarely encountered, these unusual morphologic variants of melanoma are often treacherous and may be difficult and challenging to diagnose. Thus, recognition of these variants is of paramount importance in the evaluation of any kind of epithelial or soft tissue neoplasm. Rhabdoid melanoma is one such example of these melanoma variants. Very few cases of rhabdoid melanoma have been reported, most of which were found in metastatic lesions. We wish to expand this rather sparse body of literature by reporting a case of metastatic rhabdoid melanoma arising subcutaneously in the right thigh of a 64-year-old man with a remote history of primary toe melanoma. Moreover, we present a comparative review of the clinical, histologic, immunohistochemical and ultrastructural features of previously reported cases. We found significant heterogeneity in the extent of rhabdoid change, expression of melanocytic and non-melanocytic markers and ultrastructural filamentous patterns in the cases we reviewed. It is our hope that this case report and literature review will help pathologists in the evaluation of neoplasms with rhabdoid morphology.     

Malignant melanoma of the buttock presenting as a Buruli ulcer

We report an unusual case of malignant melanoma clinically diagnosed as Buruli ulcer, that arose in a 13-year-old boy and presented as an ulcerated, fungating 2 cm mass on the right buttock. The tumor showed the histology and immunohistology of a malignant melanoma. We present this interesting case of malignant melanoma of soft tissue, arising in an unusual location of the body. The tumor presented with clinical features of Buruli ulcer in an area endemic for this disease as well as other tropical ulcerations. Neoplasms, including melanoma, should be considered in the differential diagnosis of Buruli ulcer in endemic areas.     

Subcutaneous T-cell lymphoma: A clinical, histopathologic, and immunohistochemical study of six cases

Recently, T-cell lymphoma localized to the subcutaneous tissue has been reported. We report the clinical, histolpgic, immunohistochemical, and molecular genetic findings in 6 patients who we believe had this peculiar T-cell lymphoma with its unique morphologic and clinical features. All patients presented with deep-seated nodules, most frequently on the extremities, and with systemic complaints of low-grade fever, fatigue, myalgias, and weight loss. In all cases, the neoplastic lymphocytic infiltrate was confined to the subcutaneous tissue, predominantly in a lobular pattern. Hemorrhage, necrosis, and rare erythrophagocytosis were also seen. Immunohistochemical staining was predominantly T-cell reactive (CD43, CD3, and CD45RO). Clonal rearrangements of the β and γ chains of the T-cell antigen receptor genes were found in 1 case. Three of the 6 patients died within 22 months of the diagnosis of lymphoma. We believe that subcutaneous T-cell lymphomas are a distinctive group of peripheral T-cell lymphomas with unusual clinical and morphologic features and that they should be distinguished from other types of lymphoma.     

Localized soft tissue angiomatosis with subsequent development of angiolymphoid hyperplasia with eosinophilia

We report an unusual case of arteriovenous (AV) malformation (localized form of soft tissue angiomatosis): a reaction of angiolymphoid hyperplasia with eosinophilia (ALHE) developed over the lesion of this pre-existing AV malformation. To the best of our knowledge, there is no other report of ALHE in association with a pre-existing bona fide AV malformation. A 71-year-old woman consulted our clinic because a reddish, dome-shaped tumor on her preauricular region, present as a slightly elevated, skin-colored tumor since her childhood, had rapidly enlarged and become more elevated with color change during the preceding two months. The histological features of most of the lesion corresponded to those of soft tissue angiomatosis (localized form). The proliferative vessels underlying the soft tissue angiomatosis streamed out and welled into the dermis with an accompanying prominent inflammatory infiltrate composed of lymphoid cells and eosinophils: these histological features were characteristic of ALHE. The recent abrupt clinical changes in the tumor were considered to represent a reaction to the angiomatosis with development of ALHE.     

A morphologic, pathologic, and virologic study of anogenital warts in men.

BACKGROUND AND DESIGN--Infection with human papillomavirus (HPV) in the anogenital region is associated with benign papillomas (condyloma acuminatum), subtle verrucous changes, subclinical infection, and malignant lesions. Although both men and women are affected, much of the investigation has been directed toward women in the study of cervical and vulvar carcinoma. The current investigation focuses on HPV infection in men. This study was undertaken to correlate the clinical spectrum of disease in our population of male patients with histopathologic features, immunoperoxidase staining for viral capsid antigen, and viral typing. Genital lesions from 26 patients were examined and tested prospectively over a 1-year period. RESULTS--The 26 lesions examined demonstrated variable morphologic features with regard to location, size, surface characteristics, and color. Histopathologic features were consistent with the diagnosis of venereal warts, but not necessarily diagnostic. Three of five standard histopathologic criteria were present in only 71% of the specimens. Despite the morphologic variability and the indeterminant histopathologic findings, 20 of 23 lesions positive for the genital tract HPV types tested contained HPV types 6 and/or 11. CONCLUSIONS--We conclude that the morphologic appearance of anogenital warts does not necessarily correlate with HPV type. Histopathologic study is helpful in excluding other diagnoses but may be indeterminant in the diagnosis of venereal warts. All men with anogenital warts should be counseled, treated, and undergo follow-up regardless of HPV type.     

CD34+ pigmented fibrous proliferations: the morphologic overlap between pigmented dermatofibromas and Bednar tumors

Pigmented dermatofibrosarcoma protuberans (DFSP; Bednar tumor) constitutes 5%-10% of all cases of DFSP and shows morphologic features that overlap with melanocytic and fibrous proliferations. We report 2 unusual cases of pigmented fibrous proliferations that demonstrate features of dermatofibromas and DFSP. The first case is that of a 19-year-old man with a 3-year history of a slowly growing pigmented lesion on the right arm. On clinical exam, the lesion was a 7-mm firm pigmented papulonodular lesion. The second case is that of a 31-year-old woman with a 4- to 5-year history of a slowly enlarging, asymptomatic "dark area" on the right buttock. On clinical exam, the lesion was a 2-cm darkly pigmented flat nodule. Morphologically, both lesions are primarily dermal proliferations of spindled cells admixed with pigmented dendritic melanocytes. The lesional cells trap collagen fibers at the periphery and there is basal cell hyperpigmentation. Adnexal structures are effaced, but significant trapping of subcutaneous fat is not present. By immunohistochemistry, both lesions show focal CD34 positivity but are negative for Factor XIIIa and melanocytic markers. Although overlap between standard dermatofibromas and DFSP is well documented in the literature, pigmented fibrous lesions with features of both entities are not well described.     

Subungual perineurioma: a peculiar location

We report the first case of subungual perineurioma presenting as clubbing of one finger, an unusual clinical feature. Histological study showed a myxoid tumour but with, unexpectedly, a diffuse expression of CD34. Differential diagnoses between perineurioma and myxoid soft tissue tumours expressing CD34 are discussed.     

Proliferating Hybrid Follicular Tumor

Solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm that typically arises in the pleural cavity but may also be seen at extra‐pleural sites including liver, lung, thymus, orbit, soft tissue, and rarely the skin. The diagnosis of cutaneous SFT is challenging due to its histologic variability and lack of distinctive immunophenotype. We report a case of SFT with unusual histologic and immunophenotypic findings. A 23‐year‐old man presented with a two‐year history of a painful, enlarging scalp mass. Histologic examination revealed a variably cellular neoplasm composed of tumor cells with uniform oval nuclei embedded in a collagenous matrix. In areas there was a prominent hemangiopericytoma‐like vasculature. The tumor was strongly immunoreactive for CD34 and had some immunoreactivity for S100. The diagnosis of SFT was rendered based upon the histologic features and strong immunoreactivity for CD34. Immunoreactivity for S100 is rarely seen in SFT. The tumor had a mitotic rate of 8/50 hpf. Mitotic rates greater than 5/50 hpf have been associated with aggressive behavior in SFTs of soft tissue. The significance of aggressive histologic attributes with clinical outcome is yet to be determined in cutaneous SFT. Cutaneous SFTs should be considered in the differential diagnosis of superficial spindle cell tumors.     

Multiple cutaneous sclerosing perineuriomas: an extensive presentation with involvement of the bilateral upper extremities

A perineurioma is a benign peripheral nerve sheath neoplasm thought to be derived from cells of the perineurium, the cellular sheathing of peripheral nerve fascicles. The histological, immunohistochemical (positive for epithelial membrane antigen, collagen type IV, laminin and vimentin but not S-100 protein) and ultrastructural features of a perineurioma form the basis for diagnosis. Two types of perineuriomas are recognized, intraneural and soft tissue (extraneural) forms. Sclerosing perineurioma is considered a variant of the soft tissue perineurioma. In all but one of the reported cases, this subtype presented as a solitary clinical lesion with a predilection for the fingers and palms of young adults. In 2002, Huang and Sung described a 16-year-old boy with one sclerosing perineurioma on each hand. The unusual case presented herein is a 21-year-old woman manifesting more than 30 cutaneous sclerosing perineuriomas on the hands and arms. This case is the most extensive presentation of sclerosing perineuriomas documented to date and extends the clinical presentation spectrum of these lesions.     

Syringolymphoid hyperplasia with alopecia

A 26-year-old man presented with an unusual skin lesion consisting of a hairless area on the left leg studded with reddish-brown papules, together with follicular hyperkeratosis and circumscribed anhidrosis. Skin biopsies showed dense lymphocytic infiltrates in the dermis and around hyperplastic sweat ducts and glands. Similar clinical and histological features have been described in two previous case reports. We propose the term syringolymphoid hyperplasia with alopecia for this unusual skin disease.     

Erysipelas after breast cancer treatment (26 cases)

Erysipelas is a bacterial hypodermal cellulitis usually associated with Streptococcal infection. Erysipelas of the upper limbs in women treated for breast cancer is relatively rare. We undertook a 10-year retrospective study identifying 26 cases of erysipelas of the upper limb following treatment for breast cancer; we describe the clinical, therapeutic, and evolutionary aspects. The age of our patients ranged from 37 to 80 years with a mean age of 53. All patients had a breast surgery and lymphadenectomy. Fifteen patients had chemotherapy and 23 had radiotherapy. The erysipelas appeared with an average of 5.23 years after cancer treatment (3 months to 15 years) and was recurrent in nine cases. Lymphedema occurred in eighteen patients. The first signs were fever and shivering in 25 patients. The clinical aspect was an inflammatory plaque. The physical findings of erysipelas included a raised edge (6 cases), blisters (1 case), purpura (1 case), and cellulitis (1 case). The portal of entry was not found in eleven patients. The upper limb was affected in all cases. Involvement of the axillary folds or the chest was observed in eight cases. Treatment with penicillin was undertaken for all patients; the length of treatment varied from 11 to 26 days. Lympadenectomy and radiotherapy in breast cancer may lead to lymphedema, which can be evident or sometimes discrete. Those patients who developed erysipelas in our series usually fared well with treatment, but many had recurrences attributed to persistent lymphedema. It was also of note that for many patients in this series, the portal of entry was not identified.     

Profound digital collagen atrophy: a new cutaneous presentation of adrenal-dependent Cushing''s syndrome

A 59-year-old Caucasian housewife presented with a 2-year history of marked loss of tissue substance from the finger and toe pulps and the heel pads. There was no clinical evidence or history of urticaria or other inflammatory change. Investigations demonstrated a raised plasma cortisol secondary to a left adrenal adenoma. Skin biopsies showed abnormalities of dermal collagen, but no evidence of elastin destruction. This case presents an unusual variant of the cutaneous atrophy associated with Cushing's syndrome.     

Infantile hemangiopericytoma--case report and literature review

Infantile hemangiopericytoma is a rare soft tissue neoplasm of pericytic origin and is almost always benign, despite its worrisome pathologic features. We describe a 2-month-old male infant with a soft tissue mass on his right thigh. Histologically, the lesion showed a characteristic hemangiopericytoma-like vascular pattern, multilobulation, and moderate mitotic activity. These morphologic features were prediagnosed as infantile hemangiopericytoma. However, immunohistochemical and ultrastructural studies revealed a heterogeneous cellular composition, primarily pericytes and endothelial cells, similar to that observed in infantile myofibromatosis.     

Cutaneous nodular amyloidosis masquerading as a foot callus

We report a unique case of a 69-year-old man who presented with a 1-year history of pink nodules and plaques limited to the anterior plantar surface of the right foot and a 30-year history of a callus on the right heel. Histologic examination findings of both areas showed deposits of amorphous, eosinophilic material and an infiltrate of plasma cells in the dermis. Congo red-stained deposits exhibited apple-green birefringence with polarized light. Results of an extensive clinical and laboratory evaluation showed no evidence of systemic amyloidosis. The diagnosis of nodular primary localized cutaneous amyloidosis (PLCA) was made. Nodular PLCA isolated to pedal or strictly plantar surfaces is an unusual presentation of PLCA. The origin and the clinical and histopathologic features of nodular PLCA are reviewed.     

Cellular angiofibroma of the vulva: a clinicopathological study of two cases with documentation of some unusual features and review of the literature

BACKGROUND: Cellular angiofibroma (CA) of the vulva is a recently described condition, whose clinical and pathological features are poorly known. METHODS: We have encountered two cases of this very unusual tumor. Their clinical and pathological features were analyzed and compared to those reported in the literature. RESULTS: Both patients were middle-aged women. In each case, the lesion had the clinical appearance of a vulvar cyst, located in the lateral aspect of the clitoris and the right labium majus, respectively. Microscopically, the lesions were well circumscribed but not truly encapsulated. Both were composed of small spindle cells arranged in short fascicles and mixed up with relatively abundant small- or medium-sized rounded vessels. While mitotic activity was perceptible in both cases, no cellular atypia could be demonstrated. A striking feature seen in one case was the presence of pseudoangiomatous changes in the stroma, similar to those occasionally found in spindle cell lipoma. Phenotypically, the tumor cells consistently expressed vimentin, CD99, and both estrogen and progesterone receptors. A discrete CD34 or smooth muscle actin immunoreactivity was also found in one case. No expression of S-100 protein, Bcl-2 protein, CD117 (c-kit gene product), epithelial membrane antigen, desmin, or h-caldesmon could be demonstrated. CONCLUSION: This study further illustrates that CA of the vulva has distinct clinical and pathologic features that set it apart from the other soft tissue conditions involving this area. However, like many soft tissue neoplasms, this tumor also exhibits some variation in its histological or immunohistochemical features.     

Primary hydatid cyst in the soft tissue of the face: an exceptional occurrence

To emphasize that solitary hydatid cyst can be localized in the soft tissue and present as a soft tissue mass even in an unusual site like face, we report the case of a 42-year-old male patient presenting with a slowly growing mass in right temporal region. Computed tomography (CT) scan showed an encapsulated mass with multiple cysts. Histopathological examination revealed the characteristic findings, which were consistent with soft-tissue hydatid disease. In the absence of visceral organ involvement, this is the first reported case of a primary subcutaneous hydatid cyst in the skin of face in India. In the English literature, only one case of this kind has been reported till date. When imaging methods confirm cystic nature of a swelling, even in unusual sites, one should always keep a possibility of hydatid cyst and manage accordingly during surgery to avoid precipitation of acute anaphylaxis.     

Primary cutaneous marginal zone lymphoma with subclinical cutaneous involvement and biclonality

Primary cutaneous extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) represents a monoclonal B-cell neoplasm that typically presents with papules, plaques or nodules. We describe a patient with a primary cutaneous MALT lymphoma with unusual clinical features and an unusual immunophenotype. Conventional microscopy together with immunohistochemistry and in-situ hybridization showed the presence of lymphoma in normal-appearing and minimally erythematous skin as well as in clinically involved skin. Furthermore, at least two distinct clones were shown, one of which had κ-light chain restriction, and the other of which had λ-light chain restriction. This case represents a newly described clinical appearance of primary cutaneous MZL and shows that some patients may have more than one neoplastic clone.     

Primary giant cell tumor of soft tissue of the groin – a case of 46 years duration

Background: Soft tissue giant cell tumor (GCT-ST) of low malignant potential is an uncommon neoplasm, considered the soft tissue counterpart of giant cell tumor of bone. GCT-ST mainly affects young to middle-age adults and presents as a painless growing mass mainly located in the lower extremities and trunk. Histologically, this tumor is characterized by a mixture of uniformly scattered osteoclast-like multinucleated giant cells intimately admixed with short fascicles of spindled cells. Complete excision with negative surgical margins is associated with a benign clinical course in most cases.
Methods: The authors report the clinicopathological and immunohistochemical features of an unusual GCT-ST of 46 years duration previously histologically misdiagnosed as Kaposi's sarcoma.
Results: Histologically, the tumor was characterized by a multinodular growth pattern with osteoclast-like multinucleated giant cells admixed with spindle cells partially arranged in a storiform pattern, fibrosis and foci of haemorrhage and mature bone. Immunohistochemistry revealed CD68 reactivity of the multinucleated giant cells.
Conclusion: GCT-ST is a rare neoplasm characterized by benign clinical course if excised adequately, as shown by our case of exceptionally long duration. Emphasis is placed on the importance of differential diagnosis with other giant cell-rich soft tissue neoplasms because clinical behaviour, prognosis and treatment significantly differ.