Department of Oral Pathology, Amrita School of Dentistry, AIMS Campus, AIMS Ponekkara (P.O), Kochi, Kerala, 682041, India.
Head Neck Pathol. 2021 Dec;15(4):1313-1321. doi: 10.1007/s12105-020-01265-6. Epub 2021 Jan 4.
Carcinoma cuniculatum is a locally aggressive, low-grade variant of squamous cell carcinoma. Though primarily a tumour of the skin, it has also been found to be arising from various mucosal surfaces including oral mucosa. When it occurs in the mandible it starts as a mucosal lesion which progressively invades the jaw and destroys the underlying bone, often presenting as a cystic lesion. With only around 30 cases reported in the mandible, this entity remains an elusive one for the pathologist and all except a few cases reported so far, had been misdiagnosed initially. The delay in the diagnosis is a major concern as it results in further progression of the disease with extensive involvement of bone and associated soft tissue structures. The aim of this article is to discuss the histopathological challenges faced in arriving at the diagnosis of a case of carcinoma cuniculatum involving the mandible. Owing to its overlapping clinico-pathological characteristics with odontogenic keratocyst the lesion has been misdiagnosed initially resulting in inadequate management, leading to multiple recurrences and unexpected complications. As a few cases like the present one had been erroneously diagnosed as odontogenic keratocyst previously, it becomes important to identify the varied clinical presentation and unique histopathological characteristic of carcinoma cuniculatum of mandible to help us in recognizing this often-missed entity at an earlier stage, so that it can be managed effectively.
腺样囊性癌是一种具有局部侵袭性的低级别鳞状细胞癌变异型。虽然它主要是一种皮肤肿瘤，但也有报道称它起源于各种黏膜表面，包括口腔黏膜。当它发生在下颌骨时，它首先是一种黏膜病变，逐渐侵犯颌骨并破坏下方的骨骼，通常表现为囊性病变。由于在下颌骨中仅有约 30 例报道，这种实体对于病理学家来说仍然是一个难以捉摸的问题，除了迄今为止报道的少数病例外，最初都被误诊。诊断的延迟是一个主要问题，因为它会导致疾病的进一步进展，广泛涉及骨骼和相关的软组织结构。本文的目的是讨论在诊断涉及下颌骨的腺样囊性癌病例时所面临的组织病理学挑战。由于其与牙源性角化囊肿的临床病理特征重叠，该病变最初被误诊，导致管理不当，导致多次复发和意外并发症。由于像目前这样的少数病例之前曾被错误地诊断为牙源性角化囊肿，因此，重要的是要识别下颌骨腺样囊性癌的不同临床表现和独特的组织病理学特征，以帮助我们在更早阶段识别这种经常被忽视的实体，从而可以有效地进行管理。
