Neoplasm of uncertain behavior of endocrine glands (D44)

The ICD-10 code section D44 covers neoplasms of uncertain behavior specifically affecting the endocrine glands. These codes are used when a tumor is present in glands such as the thyroid, adrenal, parathyroid, or pituitary, but its malignant potential is not clearly determined.

Within this section, specific codes like D44.0 identify tumors in the thyroid gland, often referred to as neoplasms of the thyroglossal duct or uncertain behavior of the thyroid gland. Codes D44.1 through D44.12 cover adrenal gland tumors, including distinctions between right, left, or unspecified locations, which may correspond to terms like adrenal cortex or medulla tumors. Codes such as D44.2 and D44.3 address uncertain neoplasms of the parathyroid and pituitary glands, respectively, including variants like pituicytoma or craniopharyngioma (D44.4). Other rare neoplasms involving the pineal gland (D44.5), carotid body (D44.6), and aortic body or other paraganglia (D44.7) are also captured here. For tumors of unspecified endocrine glands, D44.9 is used. These ICD-10 codes assist healthcare coders in accurately documenting tumors whose exact nature; benign or malignant; is uncertain, facilitating proper clinical tracking and management.

Instructional Notations

Type 1 Excludes

A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.

  • multiple endocrine adenomatosis E31.2
  • multiple endocrine neoplasia E31.2
  • neoplasm of uncertain behavior of endocrine pancreas D37.8
  • neoplasm of uncertain behavior of ovary D39.1
  • neoplasm of uncertain behavior of testis D40.1
  • neoplasm of uncertain behavior of thymus D38.4

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Carotid Artery, Common

The two principal arteries supplying the structures of the head and neck. They ascend in the neck, one on each side, and at the level of the upper border of the thyroid cartilage, each divides into two branches, the external (CAROTID ARTERY, EXTERNAL) and internal (CAROTID ARTERY, INTERNAL) carotid arteries.

Carotid Body Tumor

Benign paraganglioma at the bifurcation of the COMMON CAROTID ARTERIES. It can encroach on the parapharyngeal space and produce dysphagia, pain, and cranial nerve palsies.

Craniopharyngioma

A benign pituitary-region neoplasm that originates from Rathke's pouch. The two major histologic and clinical subtypes are adamantinous (or classical) craniopharyngioma and papillary craniopharyngioma. The adamantinous form presents in children and adolescents as an expanding cystic lesion in the pituitary region. The cystic cavity is filled with a black viscous substance and histologically the tumor is composed of adamantinomatous epithelium and areas of calcification and necrosis. Papillary craniopharyngiomas occur in adults, and histologically feature a squamous epithelium with papillations. (From Joynt, Clinical Neurology, 1998, Ch14, p50)

Empty Sella Syndrome

A condition when the SELLA TURCICA is not filled with pituitary tissue. The pituitary gland is either compressed, atrophied, or removed. There are two types: (1) primary empty sella is due a defect in the sella diaphragm leading to arachnoid herniation into the sellar space; (2) secondary empty sella is associated with the removal or treatment of PITUITARY NEOPLASMS.

Glomus Jugulare Tumor

A paraganglioma involving the glomus jugulare, a microscopic collection of chemoreceptor tissue in the adventitia of the bulb of the jugular vein. It may cause paralysis of the vocal cords, attacks of dizziness, blackouts, and nystagmus. It is not resectable but radiation therapy is effective. It regresses slowly, but permanent control is regularly achieved. (From Dorland, 27th ed; Stedman, 25th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, pp1603-4)

Glomus Tympanicum Tumor

A rare PARAGANGLIOMA involving the GLOMUS TYMPANICUM, a collection of chemoreceptor tissue adjacent to the TYMPANIC CAVITY. It can cause TINNITUS and conductive hearing loss (HEARING LOSS, CONDUCTIVE).

Paraganglioma

A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992)

Paraganglioma, Extra-Adrenal

A relatively rare, usually benign neoplasm originating in the chemoreceptor tissue of the CAROTID BODY; GLOMUS JUGULARE; GLOMUS TYMPANICUM; AORTIC BODIES; and the female genital tract. It consists histologically of rounded or ovoid hyperchromatic cells that tend to be grouped in an alveolus-like pattern within a scant to moderate amount of fibrous stroma and a few large thin-walled vascular channels. (From Stedman, 27th ed)

Pinealoma

Neoplasms which originate from pineal parenchymal cells that tend to enlarge the gland and be locally invasive. The two major forms are pineocytoma and the more malignant pineoblastoma. Pineocytomas have moderate cellularity and tend to form rosette patterns. Pineoblastomas are highly cellular tumors containing small, poorly differentiated cells. These tumors occasionally seed the neuroaxis or cause obstructive HYDROCEPHALUS or Parinaud's syndrome. GERMINOMA; CARCINOMA, EMBRYONAL; GLIOMA; and other neoplasms may arise in the pineal region with germinoma being the most common pineal region tumor. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p2064; Adams et al., Principles of Neurology, 6th ed, p670)

Sella Turcica

A bony prominence situated on the upper surface of the body of the sphenoid bone. It houses the PITUITARY GLAND.