Pilomatrixoma with Atypical Features: A Case Report

Pilomatrixoma is an uncommon, benign tumor with differentiation towards both the hair matrix and cells arising in the cortex, most frequently appearing in the first or second decade of life. In rare instances, pilomatrixomas can show malignant transformation. Pilomatrix carcinoma is extremely uncommon and has traditionally been considered a tumor of low malignant potential; however, a high local recurrence rate has been reported. There is a paucity of literature on these lesions, with only a few reports describing the spectrum of malignant changes seen in these lesions. In this case report, we present a case of pilomatrixoma in an adult patient showing atypical features. While the tumor is small, there are focal features that suggest progression to malignancy, but do not fulfill the criteria for pilomatrix carcinoma. These focal atypical features include a focal infiltrative pattern at the periphery, with a variable cytological atypia and an increased mitotic rate, up to five mitotic events/high-power field. Irregular foci of central necrosis (comedonecrosis) were present in several lobules. Some of the features identified were similar to a subset of pilomatrixoma, known as “proliferating pilomatrixoma.” However, our case did not have the diffuse changes or larger size that has been frequently reported in “proliferating pilomatrixoma.” In conclusion, given the lack of focality of the changes, the lesion in our case is best described as a pilomatricoma with atypical features. Furthermore, our case may highlight the need to ensure close clinical follow-up for these lesions with unexpected atypical features that raise concern of recurrence and malignant transformation.

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RARE-31. TREATMENT OUTCOME OF CHILDREN WITH PLEOMORPHIC XANTHOASTROCYTOMA: RETROSPECTIVE ANALYSIS – A SINGLE INSTITUTION EXPERIENCE

Neuro-Oncology ◽

10.1093/neuonc/noz175.954 ◽

2019 ◽

Vol 21 (Supplement_6) ◽

pp. vi228-vi228

Author(s):

Ossama Maher ◽

Toba Niazi ◽

Ziad Khatib ◽

John Ragheb

Keyword(s):

Local Recurrence ◽

Retrospective Analysis ◽

Malignant Transformation ◽

Cranial Nerve ◽

Pleomorphic Xanthoastrocytoma ◽

Braf V600e ◽

Biological Behavior ◽

High Local Recurrence Rate ◽

Cranial Nerve Palsies

Abstract BACKGROUND Pleomorphic xanthoastrocytoma (PXA) and anaplastic pleomorphic xanthoastrocytoma (APXA) are two types of rare astrocytomas in pediatrics. There is limited literature reporting their outcomes. METHODS A retrospective analysis of pediatric patients with diagnosis of PXA and APXA treated at Nicklaus Children’s Hospital was conducted using descriptive measures. RESULTS A chart review of patients with brain tumors from 2001 to 2019 revealed that 13 patients (median age 6 years, range 2–17 years) were diagnosed with non-metastatic PXA (n=12) and APXA (n=1). Six patients (46%) were male. Clinical presentation included seizures (n=8), headaches (n=2) cranial nerve palsies (n=2). Diagnostic imaging showed tumor in the temporal lobe (n=5), parietal lobe (n=2), temporoparietal lobe (n=2), frontal lobe (n=1), occipital lobe (n=1), others (n=2). BRAF V600E mutation were identified in three of five analyzed tumors including PXA (n=4) and APXA (n=1). Surgical intervention consisted of gross total resection (n=8) and near/subtotal resection (n=5). Ten patients (76%) required a second surgery (median 2; range 1–4) due to local recurrence. The median time to recurrence was 3 years (range 6 months - 7 years). Three patients (23%) had malignant transformation of PXA, which occurred three to eight years from the initial surgery; one of them also had extensive spinal metastasis. Focal radiation was given to six patients (46%) due to multiple recurrence (n=2), malignant transformation (n=3), APXA (n=1). At a median follow up of 8 years (range 3 to 20 years), eight patients remain alive, three patients had lost to follow up, two patients died from progressive disease. Long-term sequelae varied as follows; seizures (n=5), motor weakness (n=4), cranial nerve palsies (n=2), and learning disability (n=1). CONCLUSION PXA is associated with high local recurrence rate and uncommonly malignant transformation to APXA in pediatrics, which requires close follow-up due to unpredictable biological behavior of these tumors.

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Clinical, Dermoscopic and Histhopatological Findings in Diagnosis of Nevus Spilus

Health & Medical Journal ◽

10.33854/heme.v3i1.570 ◽

2020 ◽

Vol 3 (1) ◽

pp. 60-66

Author(s):

Amillia Risa ◽

Ennesta Asri ◽

Irdawaty Izrul ◽

Alimudin Tofrizal

Keyword(s):

Case Report ◽

Malignant Transformation ◽

Left Cheek ◽

Yag Laser ◽

Background Light ◽

Reticular Pattern

Introduction: Nevus spilus (NS) are seen in 0.2% to 2.3% of the population and have 0,13% to 0,2% risk for malignant transformation. Clinical, dermoscopic, and histhopatological features were describe in this case report in order to be easily recognize NS. Although NS is a benign cutaneous anomaly it has potential malignant transformation and requires regular follow up. Case Report: A case of nevus spilus in 23 years-old female was reported. There were multiple asymptomatic brownish pigmented spots over brownish patch on left cheek which gradually increased in number and size since 1 year ago. Dermatologic state: brown macules and dark brown papules in a speckled, overlying background café au lait macule. Dermoscopy revealed reticular pattern in background light brown and dark reticuloglobular pattern in dark speckled. Histopathology showed elongation of rete ridges with grouping of melanocyte cells at the tip, and proliferation of nevus cells. Conclution: Patient was planned to treat with Nd-Yag laser.

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Abriks's tumor in a teenager's tongue - a case report

Polski Przegląd Otorynolaryngologiczny ◽

10.5604/01.3001.0012.7839 ◽

2018 ◽

Vol 7 (4) ◽

pp. 1-5

Author(s):

Przemysław Krawczyk ◽

Daniel Majszyk ◽

Antoni Bruzgielewicz ◽

Kazimierz Niemczyk

Keyword(s):

Case Report ◽

Children And Adolescents ◽

Malignant Transformation ◽

Surgical Resection ◽

Granular Cell Tumor ◽

Benign Neoplasm ◽

Granular Cell ◽

Cell Tumor ◽

Upper Airways

Granular cell tumor is benign neoplasm rarely diagnosed among young children and adolescents. The tumor developed commonly within mucous membrane of upper airways, but precise etiology is not known. Treatment is based on surgical resection of tumor and intense follow up due to risk of recurrence and malignant transformation.

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Case for diagnosis

Anais Brasileiros de Dermatologia ◽

10.1590/s0365-05962012000300026 ◽

2012 ◽

Vol 87 (3) ◽

pp. 489-490 ◽

Cited By ~ 1

Author(s):

Sheila Viana Castelo Branco Gonçalves ◽

Neusa Yuriko Sakai Valente ◽

José Vitor de Oliveira Junior ◽

Daniele Loureiro Mangueira Paiva

Keyword(s):

Case Report ◽

Malignant Transformation ◽

Unknown Etiology ◽

Genital Area ◽

Long Term Follow Up

Porokeratosis is a primary keratinizing disorder of unknown etiology. This disorder is characterized by the presence of centrifugally enlarging hyperkeratotic plaques, associated with the histopathological hallmark of cornoid lamellae. Genital porokeratosis is extremely rare. No more than thirty cases have been reported in the literature, including only one case of linear porokeratosis confined to the genital area. This case report describes a patient with genital linear porokeratosis, who was successfully treated with cryotherapy. Over two years of follow-up, the lesion improved and there was no evidence of recurrence or signs of malignant transformation. Nevertheless, there is a need for long-term follow-up data on recurrence and malignant transformation.

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Leiomyosarcoma: Case report

10.1055/s-0039-1685339 ◽

2016 ◽

Author(s):

Poonam Garg

Keyword(s):

Overall Survival ◽

Case Report ◽

Treatment Strategies ◽

Mitotic Rate ◽

Definitive Diagnosis ◽

Overall Survival Rate ◽

Uterine Sarcomas ◽

Neo Adjuvant Chemotherapy ◽

Mesenchymal Tumours

Introduction: Uterine sarcomas are rare aggressive mesenchymal tumours with limited prognosis which accounts for only 2%-8% of all uterine malignancies. The most frequent type in uterine sarcomas is leiomyosarcoma (LMS) which is seen in about 60% of cases. Case Report: We report 2 cases who presentated with different symptomology. After examination and imaging modalities, definitive diagnosis was made after histopathology report. Treatment in the form of neo adjuvant chemotherapy followed by Surgery and chemotherapy/radiotherapy was given. On follow up, both patients had relapse and later they died. Conclusion: Rate of recurrence of leiomyosarcoma is high and prognosis depends upon age, grade, tumor size and mitotic rate. Overall survival rate ranges from 15% to 25% with a median survival of only 10 months. Early detection and more trials to evaluate treatment strategies can improve survival.

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Referred Pulpal Pain with Atypical Features in an Endodontically Treated Tooth due to Learned Phenomenon

Journal of Contemporary Dentistry ◽

10.5005/jp-journals-10031-1070 ◽

2014 ◽

Vol 4 (1) ◽

pp. 60-62

Author(s):

AR Vivekananda Pai ◽

Rachit Jain

Keyword(s):

Differential Diagnosis ◽

Case Report ◽

Previous Treatment ◽

Endodontic Treatment ◽

Diagnostic Dilemma ◽

Retrospective Diagnosis ◽

Atypical Features ◽

Endodontically Treated Tooth

ABSTRACT Pulpal pain does not refer to an endodontically treated tooth. Nevertheless, such a potential may exist based on a learned phenomenon due to previous treatment experiences but is rarely reported. Further, it is seldom reported to occur with atypical features. This case report is on a patient who was presented with pain in endodontically treated lower first molar (36) and recommended to undergo retreatment. However, endodontic treatment in 26 relieved the pain showing occurrence of referred pulpal pain in 36. But due to atypical referral and unknown endodontic status in 36, only uneventful follow-up confirmed the retrospective diagnosis of referred pulpal pain in 36. Pulpal pain can refer to an endodontically treated tooth based on learned phenomenon. Such a referral can also occur with atypical features and pose a diagnostic dilemma. Therefore, referred pulpal pain should be considered in the differential diagnosis of pain in an endodontically treated tooth. How to cite this article Pai ARV, Vikram M, Jain R. Referred Pulpal Pain with Atypical Features in an Endodontically Treated Tooth due to Learned Phenomenon. J Contemp Dent 2014;4(1): 60-62.

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Worsening cytology and lesion enlargement are useful indicators for malignant transformation of lobular endocervical glandular hyperplasia during follow-up: A case report

Gynecologic Oncology Reports ◽

10.1016/j.gore.2020.100571 ◽

2020 ◽

Vol 32 ◽

pp. 100571

Author(s):

Hisanori Kobara ◽

Tsutomu Miyamoto ◽

Toshiaki Otsuki ◽

Ayumi Ohya ◽

Tanri Shiozawa

Keyword(s):

Case Report ◽

Malignant Transformation

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Ameloblastic Fibrosarcoma: A Case Report and Literature Review

Brazilian Dental Journal ◽

10.1590/0103-6440201701050 ◽

2017 ◽

Vol 28 (2) ◽

pp. 262-272 ◽

Cited By ~ 2

Author(s):

João Paulo Silva Servato ◽

Paulo Rogério de Faria ◽

Cássio Vinhadelli Ribeiro ◽

Sergio Vitorino Cardoso ◽

Paulo Rogério de Faria ◽

...

Keyword(s):

Case Report ◽

Current Knowledge ◽

Immunohistochemical Analysis ◽

Cytokeratin 19 ◽

High Power Field ◽

Ki 67 ◽

Prognostic Features ◽

Regional Metastasis ◽

Ameloblastic Fibrosarcoma

Here is described a case of ameloblastic fibrosarcoma (AFS) affecting the posterior mandible of a woman who was treated surgically and recovered without signs of recurrence or metastasis after 12 years of follow-up. Tumor sections were immunostained for cell cycle, epithelial and mesenchymal markers. Immunohistochemical analysis evidenced high Ki-67 positivity in stromal cells (mean of 20.9 cells/High power field). Epithelial cells displayed strong positivity for p53, p63 and cytokeratin 19. In addition to the case report, a systematic review of current knowledge is presented on the AFS’s clinical-demographic features and prognostic factors. Based on the review, 88/99 cases were diagnosed as AFS, 9/99 as ameloblastic fibro-odontosarcoma and 2/99 as ameloblastic fibrodentinosarcoma. All these lesions displayed very similar clinical-demographic and prognostic features. Moreover, the review provided evidence that first treatment, regional metastasis, distant metastasis and local recurrence were significant prognostic values for malignant odontogenic mesenchymal lesions. Based on the findings, segregation among ameloblastic fibrosarcoma, ameloblastic fibrodentinosarcoma and ameloblastic fibro-odontosarcoma seems illogical, considering all these lesions have similar predilections and outcomes.

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Malignant Transformation Of Fibrous Dysplasia In Combination With Bone Cyst: A Case Report.

10.21203/rs.3.rs-605986/v1 ◽

2021 ◽

Author(s):

Meitao Xu ◽

Jiajia Wang ◽

Xi Zhang ◽

xuquan wang

Keyword(s):

Case Report ◽

Fibrous Dysplasia ◽

Malignant Transformation ◽

Proximal Tibia ◽

Bone Cyst ◽

Pathological Diagnosis ◽

Unicameral Bone Cyst ◽

Imaging Examination ◽

The Right

Abstract Background: Fibrous dysplasia and Simple bone cyst are all common benign lesions. A case of osteosarcoma developing from fibrous dysplasia in combination with unicameral bone cyst will be discussed. The radiologic, histologic characteristics and clinical prognostic of the patient will be described. As far as we know, this is the first case report of malignant transformation secondary to fibrous dysplasia in combination with unicameral bone cyst, which is extremely rare and worthy of clinical attention or vigilance. Case presentation: This study describes a case of a 20-years-old male who suffered a 7-years history of intermittent pain in his right proximal tibia, obvious after activity and progressively worsening. Clinical symptoms and imaging examination were consistent with the characteristics of benign primary bone tumor. He underwent a successful curettage operation. Pathological diagnosis was fibrous dysplasia in combination with unicameral bone cyst. After the curettage surgery, the pain in his right knee quickly disappeared and function activities returned to normal during a regular follow-up postoperative. However, He was diagnosed with a recurrence of fibrous dysplasia in combination with unicameral bone cyst and osteosarcoma malignant transformation by chief complaint symptoms of pain and swelling in the right proximal tibia, adequate imaging examination, and pathological diagnosis at 2 years follow-up. Then the patient undergo a limb salvage with tumor prosthesis reconstruction of the right knee. Unfortunately, multiple and unresectable lymph nodes distant metastases happened even with two weeks localized inguinal radiotherapy treatment and one course of neoadjuvant chemotherapy monthly. He eventually received cancer hospice care and died eight months after the diagnosis as malignant transformation and systemic multiple organ metastasis.Conclusions: Although malignant transformation of fibrous dysplasia in combination with unicameral bone cyst is very rare, patients with this disease should be monitored and received lifelong follow-up to obtain early detection, diagnosis and treatment to maximize the efficacy of treatment and survival time. The histological and immunohistochemical findings is very important but not enough. Further research is required to clarify the pathogenesis and prevent malignant transformation.

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