Nikolaos Katsoulas

Primary appendiceal mucinous adenocarcinoma alongside with situs inversus totalis: a unique clinical case

IntroductionMucinous adenocarcinoma is a rare neoplasm of the gastrointestinal tract and one of the three major histological subtypes of the primary appendiceal adenocarcinoma. The most common type of presentation is that of acute appendicitis and the diagnosis is usually occurred after appendectomy. The accurate preoperative diagnosis and management of the above condition represents a real challenge when uncommon anatomic anomalies such intestinal malrotation and situs inversus take place. Situs inversus totalis with an incidence of 0.01% is an uncommon condition caused by a single autosomal recessive gene of incomplete penetration in which the major visceral organs are mirrored from their normal positions.Case presentationWe present an unusual case of a 59 years old, previously healthy man presented with a left lower quadrant abdominal pain, accompanied with low fever, leukocytosis, anorexia and constipation. A chest radiograph demonstrated dextrocardia with a right side positioned stomach bubble. Both preoperative US and CT scan of the abdomen and pelvis declared situs inversus, with a characteristic thickening in its wall, appendix situated in the left lower quadrant of the abdomen. These findings reached to the diagnosis of acute appendicitis with situs inversus and a standard appendicectomy was performed. Pathologic evaluation established primary mucinous adenocarcinoma of the appendix and three months afterwards the patient underwent a subsequent extended left hemicolectomy.ConclusionIn conclusion, the occurrence of primary appendiceal mucinous adenocarcinoma along with situs inversus, definitely accounts as a unique clinical case. Even synchronous manifestation of primary mucinous adenocarcinoma of the appendix and situs inversus totalis represents an unusual anatomo-pathological entity, all physicians should be familiar having the knowledge to make an appropriate and accurate diagnosis that will lead to prompt and correct treatment.

Lymphangioma circumscriptum, angiokeratoma, or superficial vascular ectasia with epithelial hyperplasia?

We report a case of lymphangioma circumscriptum (cavernous lymphangioma with epithelial hyperplasia) in a 12-year-old girl, presenting as a papillary tumor on the right dorsal side of her tongue. Microscopic examination found cavernous vascular channels lined by a single layer of CD31(+), podoplanin-positive, CD34(-) endothelial cells that occupied the papillary lamina propria and were accompanied by epithelial hyperplasia. A review of the literature on oral vascular tumors with epithelial hyperplasia, namely, lymphangioma circumscriptum and angiokeratoma, provided information that draws into question the use of these terms.

Non-healing tongue ulcer in a rheumatoid arthritis patient medicated with leflunomide. An adverse drug event?

Leflunomide is a member of the disease modifying anti-rheumatic drugs group used as a treatment modality in active rheumatoid and psoriatic arthritis. “Oral ulcers” are reported in 3-5% of leflunomide medicated rheumatoid arthritis patients with adverse events, but they are not described in detail in the literature. We present a case of an ulcer in the tongue of a rheumatoid arthritis patient managed with leflunomide and contemplate on its pathogenesis. Key words:Leflunomide, oral ulcer, DHODH.

Atypical methotrexate ulcerative stomatitis with features of lymphoproliferative like disorder: report of a rare ciprofloxacin-induced case and review of the literature

Methotrexate (MTX) is an established immunomodulating agent used in low doses (LDMTX) to treat several autoimmune diseases. Ulcerative stomatitis (US) may be observed as a long-term LDMTX adverse effect showing a wide histopathologic spectrum. A 73-year old female presented with painful oral ulcers of 5 days duration. The patient had been under treatment for rheumatoid arthritis with LDMTX, while one week before presentation she was prescribed ciprofloxacin for a urinary infection. Histopathologic examination of a lingual ulcer revealed a polymorphous lymphohistiocytic proliferation with scattered binucleated atypical lymphocytes. Immunohistochemically, most cells were of T-cell lineage while the EBER test was negative and a diagnosis of MTX-induced reactive ulceration was rendered. MTX cessation resulted in complete resolution of the ulcers with no recurrences reported so far. The clinical and histopathologic features of MTX-induced oral ulcers are not always diagnostic and a detailed history and an extensive clinicopathologic investigation may be needed to exclude a lymphoproliferative disorder. Key words:Atypical oral ulcers, ciprofloxacin, lymphoproliferative disorders, methotrexate.

Reactive osteocartilaginous metaplasia in denture wearers: a rare trauma‐related lesion

OBJECTIVE To present a case of reactive osteocartilaginous metaplasia (ROCM) in the anterior edentulous mandibular ridge. BACKGROUND The ROCM secondary to chronic mechanical denture trauma is rare and appears as a focal sometimes painful mass on or near the crest of the edentulous alveolar ridge in long-term denture wearers. The literature review disclosed 24 cases involving more commonly the posterior portion of the mandible. MATERIALS AND METHODS An 80-year-old female was referred for the evaluation of a painful, submucosal nodule extending into the vestibular mucosa of the anterior edentulous mandibular region. Microscopically, cartilaginous regions exhibiting sparse hyperchromatic or binucleated chondrocytes transitioned into areas of ossification. RESULTS The diagnosis was ROCM. The presence of osteocartilaginous tissue displaying bizarre histopathological features can create a diagnostic dilemma. CONCLUSION Complete conservative surgical excision of this lesion has a very good prognosis. Surgical augmentation of the sharp edentulous mandibular ridges might be needed to avoid continuous irritation and possible recurrence.

Lichenoid lesions of the upper lip: a retrospective study of 24 cases

Background Lichenoid lesions of the upper labial mucosa, without other oral or extraoral manifestations seem to be rare. The clinicopathologic features of 24 such cases are presented and the pertinent literature is reviewed. Material and Methods 24 Caucasian patients that clinically presented lichenoid features on the upper labial mucosa, with or without lichenoid lesions on the adjacent gingiva, were included in the study. Clinical features were extracted from the patients’ records, while dental plaque/calculus accumulation and composite resin restorations of the adjacent teeth were recorded. Four cases where an incisional biopsy was performed were further evaluated. Results There were 8 males and 16 females, with a mean age of 62.7 years. 64.2% were under hypertensive therapy. In 13 cases gingival involvement was noticed, 16 cases exhibited calculus deposition, while 6 cases presented with composite resin fillings of the adjacent teeth. In 4 cases an incisional biopsy was performed showing features of lichenoid reaction. In 37.5% significant improvement was observed after topical treatment with corticosteroids and antimicrobial agents. Conclusions Lichenoid lesions of the upper lip may represent a distinct variety of oral lichenoid lesions, but as the number of cases reported so far is too small for definite conclusions on pathogenesis and management to be made, a long-term follow-up is mandatory. Key words:Lichenoid lesions, upper lip, oral lichen planus, lichenoid reactions.

Lichen Sclerosus of the Upper Lip: Report of a Case, Utilizing Shikata's Modified Orcein Stain, and Review of the Literature

ABSTRACT Background Lichen sclerosus is a rare chronic inflammatory disorder, affecting mainly the skin and the anogenital mucosa, while oral lesions can be the primary or the only manifestation of the disease. A rare case of oral lichen sclerosus, assessed histopathologically and histochemically, is presented, along with a thorough review of the English language literature. Methods A 32-year-old female presented an asymptomatic white patch affecting the skin and the mucosa of the upper lip, without other mucocutaneous involvement. A partial biopsy of the lesion was performed, along with the histochemical Shikata’s modified orcein stain for elastin fibres detection. A literature review was also performed, discussing the epidemiological data, clinical presentation, and treatment modalities of all published cases with oral involvement. Results The histopathological evaluation revealed the presence of acellular zone underneath the basal layer of the epithelium, accompanied by deep band-like chronic inflammation. Shikata’s modified orcein stain exhibited scarcity or loss of elastin fibres in the acellular subepithelial area. The diagnosis of lichen sclerosus was made. Topical application of corticosteroids was prescribed and resolution of the lesion was observed. Literature review revealed that oral lichen sclerosus is predominantly presented in females, as asymptomatic lesions of the lips and buccal mucosa; few cases have extraoral manifestations and topical corticosteroids are the main treatment intervention. Conclusions Despite its rarity, lichen sclerosus should be considered in the clinical and histopathological differential diagnosis of white patches of the oral mucosa. For rendering proper diagnosis, the histochemical Shikata’s modified orcein stain is a useful assessment tool.

Accumulation of basement membrane components in oral epithelial lesions

To the Editor, Eosinophilic globules at the dermoepidermal junction above the tips of the dermal papillae, known as Kamino bodies, are a hallmark of Spitz nevus and are of importance in its differential diagnosis from melanoma.1 Kamino bodies stain purple with periodic acid-Schiff after diastase treatment (PAS-D) and blue with trichrome stain but do not stain as amyloid with crystal violet.1 Immunohistochemically, Kamino bodies are positive for collagen IV, collagen VII and laminin.2,3 Therefore, they are currently considered as accumulations of basement membrane components that originate from keratinocytes and/or melanocytes of Spitz nevus.2 On the course of reevaluating the material of an immunohistochemical study4 on the expression of laminin and type IV collagen in 12 cases of oral epithelial hyperplasia (benign keratosis and/or acanthosis without dysplasia), 30 cases of oral epithelial dysplasia and 50 cases of oral carcinoma, we encountered eosinophilic globules in two cases of mild epithelial dysplasia (non-homogeneous leukoplakia, female, 81-year-old, lower lip; verrucous leukoplakia, female, 51-year-old, buccal mucosa), and a carcinoma cunniculatum (male, 79-year-old, gingiva). In all cases, serial, 5μm thick formalin-fixed and paraffin-embedded tissue sections stained with hematoxylin and eosin, PAS (without diastase), and mouse anti-human monoclonal antibodies for laminin (1:2000, LAM89; Biomakor bm, Kiryat Weizmann, Rehovot, Israel) and collagen type IV (1:50, CIV22; Dako, Holmstrup, Denmark) were available.4 Immunohistochemistry was performed with a standard avidin– biotin–peroxidase procedure (Vectastain Elite ABC kit for mouse IgG, Vector Laboratories Inc., Burlingame, CA, USA). No additional unstained sections or paraffin-embedded tissue were available for further study. In all lesions pronounced acanthosis and papillomatosis were seen. The eosinophilic globules were usually large, with scalloped borders, located intraepithelially (Fig. 1) or at the tips of the connective tissue papilla. Most globules stained purple with PAS without diastase (Fig. 1 inset), and reacted for laminin (Fig. 2 inset) and collagen IV. Rare globules stained faintly for PAS, laminin and collagen IV, and this variation in staining intensity was seen both in globules of the same lesion, as well as among those of different ones. Some globules included normal appearing or pycnotic nuclei, while melanin deposition was not identified. Occasionally, the papillary dermis was occupied by a homogeneous eosinophilic material, histochemically and immunohistochemically similar to the globules, that covered the space between the basal layer of the epithelium and the walls of capillary vessels. In step sections, the upper part of this material appeared to ‘disconnect’ from the rest and lay within the epithelium. The above described globules fulfilled most of the diagnostic criteria for Kamino bodies in Spitz nevi, i.e. they had dull eosinophilic color and scalloped borders; were located at the junction of epithelium with the lamina propria, above the tips of the papilla and not at the base of rete ridges; showed inclusion of normal appearing and pycnotic nuclei; showed positivity for PAS stain and reacted for laminin and collagen IV.1–3 However, one of the main diagnostic criteria, positivity for collagen in a trichrome stain, could not be evaluated as unstained sections or paraffin-embedded tissue were not available for further study. Therefore, a hematoxylin and eosin stained section of each case that clearly