Acquired Elastotic Hemangioma-like Change of the Vulva Associated With Lichen Sclerosus.

Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.

Incidental superficial soft tissue epithelioid angioleiomyoma.

Epithelioid angioleiomyoma (EALM) is rare in the skin and subcutaneous tissues. To the best of our knowledge, only two previous cases of this tumor have been reported. We document here the case of an 83-year-old woman who underwent complete removal of a squamous cell carcinoma of the retromolar trigone and lymph node dissection of the neck. An incidental EALM was observed in the adipose tissue. The tumor formed a unilocular, poorly demarcated neoplasm measuring 0.3 cm, and showed cavernous angiomatous spaces with villiform growth of large epithelioid cells arranged in clusters. Besides the epithelioid cells of muscular origin, bundles of well-differentiated smooth muscle cells were observed. Epithelioid cells accounted for 70% of the total. The neoplasm originated in the wall of a medium-sized vein. Epithelioid and spindle cells were positive for alpha-smooth muscle actin, calponin, h-caldesmon, and muscle-specific actin. The endothelial cells lining the vascular spaces showed intense and diffuse positivity for CD31 and ERG. The main differential diagnosis includes metastatic carcinoma, melanoma, perivascular epithelioid cell neoplasm, myopericytoma, glomangiomyoma, epithelioid glomus tumor, and epithelioid leiomyosarcoma. This report expands the morphological spectrum of the EALM. Awareness of this uncommon morphologic variant of angioleiomyoma and the use of adequate techniques can avoid misdiagnosis.

Papillary Dermal Elastosis.

Papillary dermal elastosis is a rare clinicopathological entity with only 1 case reported. The authors herein report a new case. A 38-year-old woman presented with a 10-month history of slowly progressive appearance of multiple, 1-2 mm, pruritic nonfollicular papules, located on the back. The study of the biopsy with the ordinary staining revealed numerous dermal papillae showing increased elastosis. The Verhoeff elastic stain demonstrated numerous foci of clumped and curled elastic fibers in the papillary dermis. The elastic component of the reticular dermis was normal. This case report confirms this entity and can contribute to a better understanding of the spectrum of the fibroelastolytic entities.

Histopathologic differences between cutaneous vasculitis associated with severe bacterial infection and cutaneous vasculitis secondary to other causes: study of 52 patients.

OBJECTIVES: To determine if cutaneous vasculitis (CV) associated with severe infection has some histopathologic findings that may help us to differentiate patients with this condition from other patients with CV. METHODS: We reviewed the skin biopsy specimens of patients with leukocytoclastic CV associated with a severe bacterial infection. Histopathologic findings of these patients were compared with those observed in leukocytoclastic CV secondary to other causes. Biopsy-proven leukocytoclastic CV were stratified as follows: group a): CV associated with severe underlying bacterial infection; group b): CV without severe bacterial infection but with systemic involvement; group c): CV without systemic involvement. Slides were reviewed by expert pathologists that were blind to the clinical information. The severity of vascular lesions was measured according to a semiquantitative scale (Hodge index). A comparative study between group a) and the other groups was conducted. RESULTS: group a) included 12 patients (2 women/10 men), mean age± SD 56±15 years; group b) 21 patients (10 women/11 men), 52±18 years; and group c) 19 patients (12 women/7 men), 59±24 years. Presence of neutrophilia was significantly increased in biopsies from group a) when compared with the other two groups. Also, a trend to higher frequency of pustular dermatosis was found in patients from group a). Hodge index, degree of inflammatory infiltrate and deep arterioles involvement were similar in all groups. CONCLUSIONS: Neutrophilia is common in skin biopsies of patients with CV associated with severe bacterial infection. No other histopathological findings help us to establish the presence of a severe underlying infection.

Primary intraarterial occlusive lipoma.

Lipomas are the most frequent soft tissue tumors. Intravenous lipomas are very uncommon, but even more unusual are intraarterial lipomas. A 68-year-old heavy smoker man, with chronic alcoholism, retinopathy, dyslipidemia, and a history of type 2 diabetes mellitus of more than 10 years of evolution was hospitalized in a state of dependency. He presented ulcers on both heels and right foot sole extending to the fifth metatarsal base and bedsores in the iliac and sacral regions. Ulcer cultures showed growth of Klebsiella pneumoniae OXA34. A computed tomography angiography scan revealed that the right posterior tibial artery showed several segments with signs of obstruction or sub-occlusive stenosis along its entire length, but especially in the distal two-thirds. The patient underwent supracondylar amputation of the right lower limb. Histopathological sections of the amputated leg showed calcific atherosclerosis obliterans of the posterior tibial artery and complete occlusion in the middle portion of this artery. The occlusion was due to a well-differentiated, white adipose tissue with lipid vacuoles of uniform size. To our knowledge, this case is the first known report of a primary intraarterial lipoma in a peripheral artery. The proliferating adipose tissue within the arterial lumen contributed to distal limb ischemic necrosis. Although an intraarterial lipoma is rare, it should be considered in the differential diagnosis of the causes of peripheral arterial occlusion.

Primary de novo malignant peripheral nerve sheath tumor of the breast mimicking mastitis. Report of a case and review of the literature.

Malignant peripheral nerve sheath tumor (MPNST) is a rare tumor representing <0.1% of malignant breast tumors. Here, we report a case of sporadic MPNST of the breast in a 45-year-old woman with a fast-evolving painful mass in the right breast associated with edema, redness, and increased local temperature, simulating mastitis. This presentation has not been reported. A review of the literature since 1992 has revealed 15 MPNST cases, including the present one with sufficient data for analysis. All the cases were women. The ages ranged from 16-60 years (mean 40.5 years). Duration of symptoms varied from four days to 15 years (mean 2.7 years). Tumor size ranged from 2.5-30 cm (mean 10.9 cm). 64.3% of tumors were well-circumscribed. Tumors were graded as grade 1 (7.7%), grade 2 (38.5%), and grade 3 (53.8%). Three (20%) cases showed histopathological peculiarities. Simple mastectomy was performed in four (26.7%) cases, radical mastectomy in six (40%) cases, and simple excision in five (33.3%) cases. There were no metastases in the six cases where axillary dissection was performed. Seven (53.8%) patients received adjuvant therapy, including chemotherapy and∕or radiotherapy. Local recurrence was observed in two (16.7%) patients. Distant metastasis was detected in one patient (7.7%) affecting the lung 11 months after radical mastectomy. The follow-up was available for 10 patients (mean 2.2 years, median 1 year). One patient (10%) died of the disease. At the last follow-up, 80% of patients were still alive. The main differential diagnosis includes nonpleomorphic spindle cell tumors.

Obliterative foam-cell arteriopathy. A unifying concept embracing several entities previously described as radiation, decidual, transplant, and intratumoral-associated arteriopathy.

This review article aimed to postulate the existence of a specific arterial injury having as its histological hallmark a collection of macrophages loaded with lipids in the intima of small-sized and medium-sized arteries causing narrowing or complete obstruction. The proposal is made that a series of previously described entities, such as ionizing radiation arteriopathy, acute atherosis (foam-cell decidual arteriopathy), transplant chronic arteriopathy of solid organ allografts, and intratumoral-associated foam-cell arteriopathy constitute different manifestations of the same basic morphological process identified as obliterative foam-cell arteriopathy (OFCA). OFCA is a local (single-organ) lesion in the aforementioned diverse processes with variable etiopathogenesis but converges in a single morphological marker. This arteriopathy is essentially an intimal disease. The processes in which the OFCA appears are known under a variety of names partly dependent on the location of the lesion. The basic unifying mechanism of the different entities is endothelial activation and dysfunction (local arterial endotheliopathy), preferably in small-sized or medium-sized arteries (100 to 500 µm in external diameter).

Sclerotic fibroma (storiform collagenoma)-like stroma in a fibroadenoma of axillary accessory breast tissue.

Accessory breast tissue is a subcutaneous remnant persisting after normal embryological development of the breast. It occurs most frequently in the axilla. Fibroadenomas in axillary breast tissue are rare. We report the case of a 29-year-old female patient who presented a fibroadenoma arising in the accessory breast tissue of the right axillary fossa. The neoplasm showed foci of sclerotic fibroma-like stroma. The patient had no signs of Cowden's syndrome. To the best of our knowledge, a lesion of this kind has not been previously reported. This stromal change suggests an uncommon involutional phase of the fibroadenoma with production of sclerotic and hypocellular collagen. The lesion should be differentiated from extraneural perineuroma, from the changes in fibroadenomas in Cowden's syndrome, from sclerosing lobular hyperplasia (fibroadenomatoid mastopathy) and from pseudoangiomatous stromal hyperplasia.

Subgemmal neurogenous plaques of the tongue: a systematic autopsy study.

Subgemmal neurogenous plaque (SNP) is a subepithelial nerve plexus associated with taste buds, occasionally observed in tongue biopsies. There is no evaluation of the prevalence of this structure in the general population. We present a systematic study of samples obtained at random from the dorsal portion of the oral tongue in 205 consecutive complete autopsies. Each sample was about 15 mm long and 10 mm thick. Four hundred fifty-eight samples were routinely obtained and an average of 2.23±0.88 samples per case (range 1-7) was collected. The total number of SNPs observed was 556, with a mean of 2.71±2.68 per case (range 0-16). This means that for every 15 linear mm of the oral tongue, approximately 2.7 SNPs can be present. SNPs display several ages, and they do not show sex differences. The mean size of these structures was 2.1±0.94 mm (range 0.6-3.6 mm). SNP is characterized by its unique neural, zonal pattern with a superficial neurofibroma-like area and a deeper neuroma-like area. Special features of the SNPs include the presence of taste buds (49.1%), ganglion cells (26.3%), dilated thin-walled vessels (11.3%), salivary gland excretory ducts emptying on the surface of the papillae (6.1%), moderate-severe inflammatory infiltrate (6.8%), presence of lymphoid tissue in the vicinity (7.0%), and hyperplasia of the epithelial cover with pseudoepitheliomatous appearance (7.0%). The differential diagnoses include schwannoma, neurofibroma, ganglioneuroma, traumatic neuroma, mucosal neuroma, and squamous cell carcinoma. SNPs are small, normal structures that may undergo hyperplasia and are usually seen incidentally.

Basaloid squamous cell carcinoma with prominent shadow (ghost) cell differentiation. An unreported neoplasm of the parotid gland.

Basaloid squamous cell carcinoma (BSCC) is an aggressive type of squamous cell carcinoma (SCC) predominant in the upper aerodigestive tract. To our knowledge, only one case of that tumor has been previously described in the parotid gland. Shadow (ghost) cell differentiation (SCD) is a specialized form of keratinization characteristic of pilomatricoma, and other skin tumors with follicular differentiation. SCD has also been described infrequently in some visceral carcinomas and rarely in the minor salivary glands. Recently, an SCC with prominent SCD has been reported in the parotid. We report for the first time the case of parotid BSCC with prominent SCD in an 87-year-old man. He was admitted due to the appearance in the last few months of a mass, painful on palpation, in the left parotid region. Imaging studies and tru-cut biopsies indicated the need for surgical removal. A left superficial parotidectomy, including the branch of the cervicofacial nerve, was performed. The BSCC showed predominance (62%) of SCD. Immunohistochemically, the basaloid cells were positive for ß-catenin, pan-cytokeratin (pan-CK) AE1∕AE3, CK19, high-molecular-weight CK (HMWCK), p63, p40, and cluster of differentiation 10 (CD10) in a diffuse pattern. No signs of recurrence or metastasis were observed four months after surgery. The main differential diagnoses include nuclear protein in testis (NUT) carcinoma, adamantinoma-like Ewing sarcoma, embryonal carcinoma, and basal cell adenocarcinoma of the solid type. SCD in BSCC of the parotid should be recognized to avoid misdiagnosis, especially in small samples. Although rare, BSCC with SCD should be added to the list of tumors that may originate in the parotid gland.

Pure extracellular mucin-producing (colloid) myoepithelioma of the parotid gland.

Mucinous (secretory) myoepithelioma is a very uncommon variant of myoepithelioma characterized by the presence of intracellular mucin. We herein report the findings of a 51-year-old woman who presented with a mass in the right parotid region, which had been slowly growing over the previous year. Magnetic resonance imaging revealed a rounded mass with lobulated margins in the tail of the right parotid gland that showed heterogeneous uptake in the anterior margin of the mass. A clinical diagnosis of pleomorphic adenoma was made. Superficial parotidectomy revealed a 3.6 cm encapsulated, round-shaped, light brown, gelatinous, solid tumor. The neoplasm showed a reticular pattern forming pseudoglandular structures that were filled with abundant extracellular mucin secretion. Additionally, lakes of mucin of different sizes appeared to be scattered throughout the neoplasm. The epithelioid tumor cells were medium-sized with eosinophilic cytoplasm, centrally located, small, uniform nuclei, and inconspicuous nucleoli. Mitoses were not observed. Signet-ring or univacuolated cells, ductal, and glandular components were absent. The extracellular mucin was positive for mucicarmine and Alcian blue staining. Immunohistochemical staining revealed that the tumor cells were diffusely and strongly positive for S100 protein, cytokeratin AE1/AE3, SOX10, and DOG-1. Occasional compact solid clusters of epithelioid cells showed intense positivity for CK7 and high-molecular-weight CK. The diagnosis was pure extracellular mucin-producing (colloid) myoepithelioma. To our knowledge, this type of neoplasm has not been previously published. Knowledge of this new variant will avoid diagnostic difficulties.

Nodular spindle cell proliferation in the wall of a varicose vein mimicking Kaposi's sarcoma.

Exuberant reparative reactions resembling sarcoma have been reported in the genitourinary tract, thyroid, breast, lymph node, oral cavity and skin, but not in a varicose vein. Presented herein is the case of a 55-year-old man who showed an incidental nodular lesion in the wall of a varicose vein on the left leg. The nodule consisted of fascicles of spindled cells with ovoid or elongated nuclei and delicate chromatin that showed diffuse reactivity for CD31, alpha-smooth muscle actin and D2-40. This histopathological appearance, when coupled with extravasated erythrocytes and interstitial hemosiderin deposits, resembled Kaposi's sarcoma or spindle cell angiosarcoma. Key features helpful for recognizing that the proliferation we describe is a form of tissue repair include an association with obvious hemorrhage; lack of well-formed curved fascicles of spindled cells; lack of intracytoplasmic hyaline globules; lack of intracellular vacuolization; cytological blandness; low mitotic count; absence of inmmunoreactivity for human herpesvirus-8 (HHV-8) latent nuclear antigen-1; and absence of HHV-8 in polymerase chain reaction (PCR) analysis.

Hypodermal decidualized endometrioma with aberrant cytokeratin expression. A lesion mimicking malignancy.

Decidualized endometrioma is a pseudoneoplastic lesion that may appear as a solitary nodule in the hypodermis, simulate a malignant epithelioid tumor, and can represent a diagnostic challenge. A 36-year-old woman delivered a full-term baby by cesarean. At the immediate puerperium, she complained of a subcutaneous nodule measuring 2.5 cm, underneath a previous caesarean scar from the former full-term delivery 3 years earlier. Histologic features included a nodular growth pattern of large monomorphic epithelioid cells showing diffuse positivity for cytokeratin (AE1/AE3, 18), human placental lactogen, and CD10 and focal positivity for inhibin alpha. The main differential diagnoses include trophoblastic neoplasia and deciduoid mesothelioma. Good clinicopathological correlation is essential for the correct diagnosis. Immunohistochemical stains can be misleading. An important clue is the combination of large decidualized cells and lumens lined by flat or low cuboidal cells that are atrophic endometrial glands. This lesion has a benign behavior.

Esophageal spindle cell lipoma.

Symptomatic ordinary esophageal lipomas are rare tumors. Spindle cell lipomas (SCLs) of this location are even more infrequent. To our knowledge, only a previous esophageal SCL case has been reported. We describe herein the case of a 62-year-old woman with a long history of heartburn and feeling of abdominal distension. Preoperative investigations, including a Barium meal, gastroscopy, and echoendoscopy revealed a lipomatous polypoid mass attached to the middle esophageal segment. The lesion (3.5×2×1 cm) was excised endoscopically under deep sedation. The final histopathology diagnosis was pedunculated SCL. An accurate diagnosis of esophageal SCL is crucial to rule out malignant lesions, relieve symptoms, and undertake suitable treatment. The main differential diagnosis includes well-differentiated sclerosing liposarcoma, atypical spindle cell∕pleomorphic lipomatous tumor, giant fibrovascular polyp, and fat-forming solitary fibrous tumor. Although rare, SCL should be added to the list of lipomatous tumors that can affect the esophagus. Complete excision is the appropriate treatment.

Renal oncocytoma with prominent xanthomatous reaction. A rare histopathological variant of oncocytoma.

Renal oncocytoma (RO) is a distinctive neoplasm with a well-recognized gross and cytoarchitectural appearance. However, on some occasions, it may show uncommon, atypical, or worrisome gross and microscopic features potentially generating diagnostic difficulties. We herein review the oncocytoma variant characterized by a significant intraneoplastic xanthomatous reaction that produces a variegated macroscopic appearance. This feature may pose a genuine diagnostic problem with conventional (clear cell) renal cell carcinoma (RCC) because this reaction creates a departure from the typical uniform, tan-brown appearance of oncocytoma. The microscopic presence of foamy macrophages in RO may potentially lead to diagnostic difficulties with tumors exhibiting eosinophilic cells and significant infiltration for lipid-laden foamy macrophages such as cystic RCC, unclassified RCC rich in foamy macrophages, the solid variant of papillary RCC with oncocytic features, post-neuroblastoma RCC, succinate dehydrogenase-deficient RCC, mucinous-poor tubular and spindle cell carcinoma, and the oncocytic variant of the epithelioid angiomyolipoma. In conflictive cases, an immunohistochemical panel should help to solve the diagnostic problem. Therefore, the presence of abundant foamy macrophages should not dissuade the pathologist from establishing a diagnosis of RO. Prominent xanthomatous reaction despite its low frequency (4.3%) can be considered an additional feature of RO. Thus, RO should be added to the list of renal tumors that can show a significant reaction of lipid-laden foamy macrophages. Besides, Gamna-Gandy bodies can be present in this tumor.

Incidental mesonephric remnant hyperplasia of the jejunal mesentery: A diagnostic challenge.

Mesonephric remnants are embryonic vestiges of the mesonephric (Wolffian) ducts which regress during normal development. These remnants have been uncommonly reported in the female and male reproductive tract as a spectrum of morphologic lesions that can be misdiagnosed as carcinoma. One case of mesonephric remnant hyperplasia of the jejunal mesentery incidentally found in a 47-year-old man is herein reported. This is the first description of mesonephric hyperplasia arisen in the mesentery. The presence of ducts, tubules, and cysts lined by bland, epithelial, cuboidal cells with scant cytoplasm, and diffuse pseudoinfiltrative growth pattern can raise the possibility of neoplasia. Immunohistochemically, mesonephric epithelia have a characteristic staining. CD10 highlights the apical-luminal aspect of the cells. Besides, intense reactivity is showed for high-molecular-weight cytokeratin (CK), CK7, bcl2, and vimentin. The main differential diagnosis includes mesothelial hyperplasia, epithelial mesothelioma, well-differentiated neuroendocrine tumor, and infiltration due to acinar adenocarcinoma of the prostate. However, a detailed microscopic study with the aid of immunohistochemistry helps separate mesonephric remnants from malignant processes. The mesonephric hyperplasia of the mesentery we have reported adds to the spectrum of mesonephric remnants a new location. Familiarity with this lesion is indispensable to avoid overdiagnosis.

Colonic tubular adenoma with incidental oxyntic gastric heterotopia.

Oxyntic gastric heterotopia (GH) in the colon is not common. Its presence in a colon tubular adenoma is even rare. A 73-year-old woman with a history of resected colon carcinoma underwent periodical colonoscopies for the removal of tubular adenomas for 12 years. In the last colonoscopy, a sessile, non-ulcerated polyp, centrally depressed, with a smooth surface, measuring 20 mm, located at 50 cm from the anal verge was excised. A histological study identified a tubular adenoma with focal low-grade dysplasia and ectopic gastric oxyntic epithelium. The GH, composed of parietal and chief cells, and was found incidentally. Oxyntic GH in a tubular adenoma is extraordinarily rare. To the best of our knowledge, there is only one previously published case. The main possible difficulties and∕or errors in the diagnosis include a tissue floater or a cross-contaminant. Precise diagnosis of oxyntic GH is basic for appropriate management. Diagnosis relies on histopathological examination. The immunohistochemical study for mucin 6 (MUC6) can confirm the nature of the epithelium. Oxyntic GH has the potential to produce serious complications including tumor development. However, GH is considered a benign disease and adenocarcinoma rarely occurs in the heterotopic mucosa. The optimal treatment of oxyntic GH associated with a tubular adenoma is endoscopic complete polypectomy.